Osteoarthritis: Epidemiology, Pathophysiology, Clinical Manifestations, Management PDF

Chapter 20 INTRODUCTION Connective tissue, the most widely distributed and abundant tissue in the body, is an extracellular matrix made up of ground substance (a clear viscous fluid that aids in cellular adhesion to the matrix and facilitates the movement of substances between capillaries and cell...

Chapter 20 INTRODUCTION Connective tissue, the most widely distributed and abundant tissue in the body, is an extracellular matrix made up of ground substance (a clear viscous fluid that aids in cellular adhesion to the matrix and facilitates the movement of substances between capillaries and cells), collagen, and elastic and reticular fibers. Cells, principally fibroblasts, participate in the synthesis of the matrix. Connective tissue provides support, structure, insulation, and cushion to tissues and organs of the body, as well as fills space. In connective tissue disease, the collagen and elastin are typically damaged through inflammation or immune system dysfunction. Bone, cartilage, tendons, ligaments, lymphatic tissue, and blood are all examples of connective tissue. OSTEOARTHRITIS Epidemiology Osteoarthritis is the most common form of arthritis in the United States. Affecting almost 27 million people in the United States, it is the leading cause of chronic disability in the country. It involves weight-bearing joints such as the knees, hips, feet, and lumbar spine, but it also affects the cervical spine, proximal interphalangeal joints, and distal interphalangeal joints of the hands (Fig. 20.1). Osteoarthritic involvement of the shoulders and elbows usually occurs after trauma, inflammation, or overuse. There are multiple risk factors associated with osteoarthritis, including age, especially individuals over 50 years of age, female gender, obesity, occupations that involve repetitive motions, sports activities, previous injury, muscle weakness, genetics, history of inflammatory arthritis, and other bone and joint disorders. Obesity is the single most modifiable risk factor contributing to osteoarthritis. Obesity correlates most closely to the development of knee osteoarthritis; however, it has also been shown to correlate with the development of hand osteoarthritis, indicating that obesity itself, not only increased stress on weight-bearing joints and decreased exercise, may contribute to osteoarthritis. There have been links made between certain occupations and osteoarthritis. Individuals whose job entails repetitive knee bending are prone to develop knee osteoarthritis, and individuals who perform physical labor are at an increased risk for developing hand and hip osteoarthritis. Aging is one of the most prevalent risk factors for developing osteoarthritis; only 0.1% of those ages 25 to 34 years are affected compared with 80% of individuals over age 55; however, osteoarthritis is not considered a normal process of aging. In general, females are more commonly affected by osteoarthritis than males. Osteoarthritis of the knees is more common in Black females than in White females. Osteoarthritis of the hands is most prevalent in females Pathophysiology Osteoarthritis is a disease that affects the joint as a whole because of biological, chemical, and viscoelastic changes within the joint. Tendons and ligaments are viscoelastic, meaning they lengthen while under tension but return to normal shape at rest. Cartilage, synovium, subchondral bone, synovial fluid, ligaments, periarticular muscle, and sensory nerves are altered by osteoarthritis. Cartilage is a material made primarily of water, collagen, proteoglycans, and elastin. Cartilage serves to provide joint protection by providing a smooth surface on which bones glide and disperses loads across the joint. When the cartilage is damaged from major trauma or repetitive microtrauma, osteophytes are formed by the body in an attempt to repair the damage. Osteophytes are projections of new cartilage and bone growth that form along joint lines, contributing to pain in the joint and decreased range of motion. Osteophyte formations on the proximal interphalangeal joints and distal interphalangeal joints are referred to as Bouchard's nodes and Heberden's nodes, respectively (Figs. 20.2A and B). Some of the new bone growth may break off as bone spurs and contribute to further cartilage loss. Cartilage loss is a clinical feature of osteoarthritis, causing the bone to be unprotected, which leads to the deterioration of joint function. The synovium is a membrane that lines the noncartilaginous surfaces of highly mobile joints. It produces synovial fluid, serving to lubricate the joints. In osteoarthritis, the synovial membrane may become thickened and overproduce synovial fluid, causing more pain and even greater restriction on joint movement. Chronic effusions, an overproduction of synovial fluid, may cause collateral ligaments to stretch, leading to joint laxity, or looseness, and mechanical instability, compounding joint damage. Muscles around the joints tend to atrophy as a consequence of decreased use of the joint. Radiographs may reveal sclerosis or cyst formation in the subchondral bone, which lies just below the cartilage. Ligaments, elastic band--type structures that connect bones to bones, may experience edema and fibrosis, also contributing to pain and decreased joint function Clinical Manifestations Clinical manifestations of osteoarthritis include progressive pain that increases with joint use, decreased range of motion, tenderness to touch over the joint line or around the joint, bony swelling, soft tissue swelling, deformity, and instability. Crepitus, a crackling, grating sound or feeling due to air or gas under the skin, may also be present. This is due to cartilage breakdown in the joint. Patients typically experience more pain with activity, which improves with rest Interprofessional Management Medical Management Diagnosis Diagnosis can be made based on clinical manifestations without laboratory testing or radiographs when the patient is 45 or older and presents with persistent usage-related pain in several joints and morning stiffness that lasts less than 30 minutes. The American College of Rheumatology (ACR) developed criteria for the diagnosis of osteoarthritis of the hand, hip, and knee initially in 1986. They are still current today Laboratory Testing Laboratory testing may be performed to rule out other diagnoses, such as rheumatoid arthritis (RA). Laboratory testing should also be conducted as a part of the medical management of osteoarthritis in order to monitor for side effects related to medication use. Radiographs Plain radiographs of the affected joints, such as the hands, hips, knees, and spine, along with a history and physical examination can be used to confirm the diagnosis of osteoarthritis. Evidence of osteoarthritis includes joint-space narrowing, subchondral sclerosis or cysts, and the presence of osteophytes. Plain radiographs, however, may not show evidence of osteoarthritis until the disease is well advanced. Treatment Ideally, both pharmacological and nonpharmacological therapies should be utilized to treat osteoarthritis. Unfortunately, there is no therapy available to stop the progression of osteoarthritis. The goals of care include decreasing pain and improving or maintaining joint mobility while avoiding the toxic effects of pharmacological therapy. Nonpharmacological Therapies Nonpharmacological therapies for osteoarthritis include the following: weight loss if indicated; heat and cold compress applications; aerobic exercise; physical therapy, including range of motion and muscle-strengthening exercises; the use of ambulatory assistive devices; appropriate footwear, such as lateral-wedged insoles; occupational therapy; joint protection; energy conservation; and assistive devices for activities of daily living. Also important is patient education about self-management programs and social support. Goal-setting is an important part of self-management for patients with osteoarthritis. Nurses can help patients set realistic short- and long-term goals. Pharmacological Therapy Pharmacological therapy includes acetaminophen (Tylenol), which is used in patients with mild to moderate pain related to osteoarthritis. It is safe to use in doses not exceeding 4 g daily, but be sure to ask the patient about any history of liver disease. Acetaminophen works by inhibiting prostaglandin synthesis, a modulator of the inflammatory response, and blocking the generation of pain impulses. Patients with more severe pain or those who are refractory to acetaminophen are treated with NSAIDs, which nonselectively inhibit cyclooxygenase, an isoenzyme responsible for the production of the prostaglandins. Nonselective means inhibition of prostaglandin's role in inflammation and pain but also inhibition of the gastrointestinal (GI) protective function of prostaglandin, hence the adverse effect of GI bleeding and ulceration with NSAID use. Patients with few joints affected may be treated with topical NSAIDs, such as topical diclofenac, which have similar efficacy as oral NSAIDs and a better safety profile. Osteoarthritis refractory to acetaminophen and NSAIDs may warrant treatment by using intra-articular injections of corticosteroids or hyaluronans. Intra-articular steroid injections strategically place the steroid into the joint space. The steroid inhibits the inflammatory process by suppressing the migration of polymorphonuclear leukocytes. Hyaluronans work inside the joint to provide lubrication to the articular surfaces. It is acceptable and safe to use acetaminophen, NSAIDs, and intra-articular medications in conjunction with each other; however, patients should not use more than one NSAID concomitantly because of increased risk of bleeding, development of gastric ulcers, and risk of renal damage (see Safety Alert and Geriatric/Gerontological Considerations). Opioid analgesics are an option but should be used sparingly because they may be habit forming. They bind to opioid receptors in the central and peripheral nervous systems, which decreases pain perception Complications Possible complications of osteoarthritis include chronic pain, decreased function, and toxic effects of medications. Osteoarthritis can also negatively affect comorbid conditions such as diabetes and heart failure related to a decreased ability to exercise. Surgical Management Surgical interventions may be necessary for some patients suffering from disability and severe pain related to osteoarthritis. These procedures include arthroscopic irrigation and/or débridement, arthroscopic synovectomy, surgical fusion, and total joint replacement. Arthroscopic irrigation and/or débridement is a procedure in which the joint is irrigated and expanded in order to visualize the joint and remove debris that could be promoting joint inflammation. Synovectomy is a surgical procedure used to remove excessive growth of the synovial membrane in order to reduce joint inflammation. Surgical fusion is a procedure performed to fuse together the joint surfaces to eliminate any movement of the joint. Total joint replacement involves surgically replacing the joint surface with a prosthesis Nursing Management Assessment and Analysis Clinical manifestations of osteoarthritis are related to pain and decreased function of the affected joints. In addition, there may be clinical manifestations related to pharmacological therapy. The nurse may identify the following: Unsteady gait Bony enlargement or swelling of affected joints Fatigue Painful range of motion of affected joints Elevated serum creatinine secondary to NSAID use Elevated liver enzymes related to multiple medication use Constipation secondary to decreased physical activity and/or use of narcotic analgesics Nursing Diagnoses/Problem List Pain related to the disease process Ineffective sleep patterns related to pain Self-care deficit related to decreased range of motion Impaired mobility Nursing Interventions Assessments Vital signs Hypertension may occur related to chronic NSAID therapy that alters renal function, reducing sodium excretion and enhancing fluid retention. Weight The use of NSAIDs may alter renal function as noted previously, leading to weight gain. Skin integrity Skin breakdown may occur in bony prominences because of decreased physical mobility. Serum creatinine The use of NSAIDs may lead to renal impairment by inadvertently blocking essential prostaglandins, which maintain blood flow to the kidneys, resulting in an increase in serum creatinine, while blocking the targeted prostaglandins, which are necessary for pain control. Actions Administer analgesic and anti-inflammatory medications as ordered. Acetaminophen and NSAIDs reduce pain by inhibiting prostaglandin production, thus blocking the generation of pain impulses and inhibiting the inflammatory response. Opioids alter the perception of pain. Provide cold packs for painful joints. Cold reduces inflammation. Provide a heat pad for painful muscles. Heat relaxes muscles and causes vasodilation, which improves blood flow and promotes healing. Teaching Take medications only as prescribed. Misuse or overuse of analgesics and anti-inflammatories may lead to side effects and can be dangerous; NSAID therapy may increase the risk of myocardial infarction (MI), GI bleeding, stomach ulcers, renal insufficiency, and abnormal platelet function. See Safety Alert. Report chest pain, abdominal pain, abnormal bleeding, and blood in the stool or emesis. Signs of NSAID toxicity or MI Participate in regular physical activity. Physical activity promotes good health, weight management, joint mobility, muscle strength, cardiac health, and self-efficacy. It also can reduce stress, anxiety, and depression. Assist with referring the patient to occupational and physical activities. Fitting for assistive devices, splints, and other assistive devices should be done by physical and occupational therapists. Physical and occupational therapies should be utilized to improve or maintain joint function. Assist with referring the patient to orthopedic surgery when necessary. Surgery may be necessary for end-stage osteoarthritis once other treatments have failed. Assist with home healthcare referral. Home care may be utilized for a number of reasons, including assessing patients' homes for safety hazards and teaching patients and their families about the management of osteoarthritis. Evaluating Care Outcomes It is important for patients with osteoarthritis to maintain function. A well-managed patient has pain under control and has good, unrestricted movement. This may be achieved by utilizing both pharmacological and nonpharmacological modalities. Patient compliance with therapy is best achieved when they and their support system are well educated and understand why therapies are prescribed and the possible negative outcomes related to nonadherence. RHEUMATOID ARTHRITIS Epidemiology Rheumatoid arthritis (RA) affects approximately 1% of the population. Female individuals are three times more likely to be affected than male individuals, and it is more prevalent in certain ethnic groups, such as Pima and Chippewa Native American tribes. The onset of RA may occur at any age but is most common in the third through fifth decades, and the incidence increases after the sixth decade. The exact etiology of RA is unclear; however, it is well understood that genetics and environmental factors play a key role in the development of the disease. First-degree relatives of patients with RA are at a 1.5-fold higher risk than the general population. Environmental factors such as cigarette smoke, bacteria, and viruses have been implicated as initiating factors for the development of RA in the genetically predisposed population. Some patients initially diagnosed with RA may experience long-term remission within 1 year of onset. For those patients who do not enter remission, about 60% will be disabled within 10 years. Pathophysiology Rheumatoid arthritis is a chronic, systemic, autoimmune inflammatory disease characterized by an inflammatory process that affects diarthrodial, or freely moving, joints, causing pain and swelling. The involved joints are usually distributed in a symmetrical fashion, meaning bilateral wrists, ankles, or knees (Fig. 20.4). Rheumatoid arthritis primarily targets the synovial membrane. The exact pathophysiology is unclear; however, it is known that an unknown antigen triggers an immune response, leading to synovial tissue damage. The immune system essentially fails to distinguish "self" from "non-self" and causes destruction to the synovium of joints. Inflammation of the synovium may lead to a dramatic increase in synovial fluid, impairing movement and causing pain. The synovial membrane becomes thickened and promotes the destruction of the joint (Fig. 20.5). In this process, antigens (substances that trigger an immune response in order to rid the body of that substance) activate monocytes and T lymphocytes; then immunoglobulin antibodies form immune complexes with antigens. Phagocytosis of the immune complexes generates an inflammatory reaction. Leukotrienes and prostaglandins are produced as a result of phagocytosis. Leukotrienes attract additional white blood cells, and prostaglandins modify inflammation. Collagenase, an enzyme that breaks down collagen, is also produced by leukotrienes and prostaglandins, leading to edema, proliferation of synovial membrane and pannus formation (a layer of vascular fibrous tissue), destruction of cartilage, and erosion of bone. Less commonly, there is inflammation in other organs, including the lungs, heart, skin, kidneys, blood vessels, salivary glands, bone marrow, and nervous system, leading to vasculitis, lung fibrosis, neuropathy, and kidney disease, to name a few. Clinical Manifestations Rheumatoid arthritis should be suspected in adults with inflammatory polyarthritis. Clinical manifestations include joint pain, joint swelling, erythema, morning stiffness, and fatigue. Onset is often insidious, with vague complaints of joint and muscle pain that evolves into joint pain with synovitis, inflammation of the synovial membrane, and can lead to joint destruction and deformity. Rheumatoid arthritis, if left untreated or inadequately treated, leads to irreversible joint damage and disability. Common joint deformities include swan-neck deformity caused by hyperextension of the proximal interphalangeal joints, boutonnière deformity caused by abnormal flexion of the proximal interphalangeal joints, and ulnar deviation caused by the lateral deviation of the phalanges (Fig. 20.6). Rheumatoid nodules may be formed in subcutaneous tissue over bony prominences. The nodules are usually mobile and nontender. Extra-articular (outside of the joint) clinical manifestations of RA include osteopenia (decreased bone density), muscle weakness, episcleritis (red, painful inflammation of the episclera without discharge), scleritis (inflammation of the sclera, which produces deep ocular pain), pleuritis (inflammation of the lining surrounding the lungs), pleural effusion (excess fluid accumulation around the lungs), pericarditis (inflammation of the fibrous lining that surrounds the heart), an enlarged spleen, and anemia. For a comparison with osteoarthritis, see Table 20.2. Interprofessional Management Medical Management Diagnosis Rheumatoid arthritis is diagnosed by combining clinical manifestations, laboratory values, and radiographs or ultrasound. The ACR and the European League Against Rheumatism have worked together to update criteria for diagnosing RA in order to diagnose the disease at an early stage. The criteria assign point values to joint involvement, laboratory values, and duration of symptoms. When evaluating clinical manifestations, patients typically present with peripheral joint pain, which is usually symmetrical, and complain of morning stiffness lasting greater than 30 minutes. Symptoms persisting 6 weeks or longer are consistent with RA. Another important finding of RA is synovitis. Tenderness and synovitis are assessed by palpating each joint individually. Tenderness is subjective, whereas synovitis is objective. The clinician is usually able to detect the synovitis on examination via palpation or, if not detected by palpation, through ultrasonography. Laboratory Testing Laboratory testing is not conclusive but is done to help with diagnosis and to monitor for side effects related to medication use. The presence of the antibodies, rheumatoid factor, and anticyclic citrullinated peptides may assist in the confirmation of the diagnosis, but the absence of these does not exclude the diagnosis because approximately 25% to 30% of patients are negative for antibodies. Also, a positive rheumatoid factor does not always indicate the presence of RA. Other autoimmune diseases may have a positive rheumatoid factor. Acute-phase reactants such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) may be used to detect inflammation but are not specific for RA. Elevated CRP and/or ESR is an indicator that can be useful in distinguishing inflammatory from noninflammatory arthritis. Obtaining information regarding renal and hepatic functions is necessary before initiating therapy because many medications used to treat RA can be toxic to the kidneys and liver. Baseline complete blood counts are necessary because antirheumatic medications may cause cytopenia, a reduced amount of blood cells. Common cytopenias include thrombocytopenia, a reduced number of platelets; leukocytopenia, a reduced number of white blood cells; and anemia, a reduced number of red blood cells. An elevated platelet count may indicate the presence of inflammation. Before the initiation of most biological therapies, an option for treatment of RA, a tuberculin skin test and hepatitis testing should also be performed because some biological therapies have the potential to reactivate dormant infections. Radiographs Conventional radiographs are used to assess for bony erosions and joint-space narrowing. Radiographs may be repeated during treatment to assess for disease progression and efficacy of pharmacological therapy. Magnetic resonance imaging (MRI) may detect erosions not detected by conventional radiographs. Ultrasonography may also be used to assess for synovitis and erosions not detectable by plain radiographs and is a good option for patients who decline MRI because of cost or claustrophobia. Treatment Nonpharmacological Therapy Education is very important in the management of RA. Patients must understand their disease and the measures they can take to help manage the disease. Range-of-motion exercises promote joint mobility, reduce stiffness, and improve muscle strength. Aerobic exercise promotes cardiac health. Physical and occupational therapies may be necessary to teach patients appropriate exercises and how to protect their joints, evaluate the need for assistive devices, and teach proper use of the devices. Proper nutrition is important to maintain good health, prevent obesity, and decrease the risk of heart disease. It is important for patients to take rest periods to manage fatigue and joint pain. Pharmacological Therapy The goal of pharmacological treatment is to control the inflammation that leads to joint and tissue destruction, decreasing joint pain, synovitis, and stiffness, as well as maintaining joint function and preventing joint destruction. Treatment decisions are based on disease severity and response to medications. Initial treatments include analgesics, NSAIDs, and glucocorticoids. Analgesics, including acetaminophen and narcotic agents, provide only pain relief with no alteration in the disease process. Anti-inflammatory medications provide pain relief and some reduction in inflammation but do not alter the disease process. Glucocorticoids such as prednisone may be given orally, intramuscularly, intra-articularly, or IV and can actually suppress inflammation and alter the disease process but are not safe at high doses over long periods of time. Patients with RA whose disease does not respond very well to a combination of analgesics, anti-inflammatory agents, and low-dose prednisone are treated with disease-modifying antirheumatic drugs (DMARDs). Synthetic DMARDs include but are not limited to methotrexate (Rheumatrex), leflunomide (Arava), hydroxychloroquine (Plaquenil), sulfasalazine (Azulfidine), and tofacitinib (Xeljanz). Each alters the immune system in various ways, altering the inflammatory response to decrease inflammation and slow disease progression. These medications may be used alone as monotherapy or in conjunction with one another. Before starting DMARD medications, patients should inform their healthcare provider of a history of or exposure to active tuberculosis. Many patients respond well to methotrexate, which has been accepted as a first-line DMARD of choice to treat RA. Methotrexate is taken once weekly orally, subcutaneously, or intramuscularly (see Safety Alert). Traditionally, patients move on to additional or alternative medications if methotrexate alone does not control the disease (Table 20.3). Over the last decade, several medications, known as biological therapies, genetically engineered medications made from living organisms and their products, such as proteins, have obtained approval by the U.S. Food and Drug Administration to treat RA. These medications have proven to be beneficial in slowing or even halting the progression of RA. To date, the approved biological therapies must be infused IV or injected subcutaneously. Biological therapies target specific entities of the inflammatory cascade, thus interrupting the immune response and decreasing inflammation. Surgical Management Surgical interventions including joint replacement or fusion may be necessary to help alleviate pain but may not actually improve function. Patients may choose to have surgical removal of rheumatoid nodules, but they may return over time. Complications Many complications may be associated with RA, which may be caused by uncontrolled disease or as a result of pharmacological therapy. Patients can develop decreased function of joints and permanent joint deformities, which may lead to permanent disability if the disease is not well controlled. Extra-articular complications include increased risk of infection and increased risk of developing cancer. Nursing Management Assessment and Analysis As with osteoarthritis, clinical manifestations of RA are related to pain and decreased function of the affected joints. In addition, there may be clinical manifestations related to pharmacological therapy. The nurse may identify the following: Unsteady gait Bony enlargement or swelling of affected joints Warmth and redness of joints Painful range of motion of affected joints Increased incidence of infection Elevated serum creatinine secondary to NSAID use Elevated liver enzymes secondary to methotrexate or leflunomide Constipation secondary to decreased physical activity and/or use of narcotic analgesics Nausea and oral ulcers related to methotrexate use Cough and/or shortness of breath due to interstitial lung disease, which can be caused by RA or by methotrexate therapy Self-care deficit Fatigue Nursing Diagnoses/Problem List Pain related to disease process Ineffective sleep patterns related to pain Self-care deficit related to decreased range of motion Impaired joint mobility and function Nursing Interventions Assessments Joint pain and mobility Joint pain and mobility are indicators of treatment efficacy and disease progression. Temperature An increase in temperature is an indicator of infection. Laboratory testing CRP and ESR Elevated values are indicative of inflammation. Hemoglobin May be less than normal in patients with active RA, but a decreased value may also indicate a GI bleed, which can be caused by NSAID use. Serum albumin Decreased value is correlated with increased disease severity because it is a negative acute-phase reactant and decreases in response to acute inflammation. Platelet count May be elevated in very active disease because inflammation causes elevated platelet count. Liver and renal function Elevated levels may be due to medications used to treat RA. Assess for pleural effusion, pericarditis, pleuritic, scleritis, episcleritis, and osteopenia. Extra-articular clinical manifestations of RA may be present due to the inflammatory changes outside of the joints. Actions Administer analgesics and anti-inflammatories as ordered. Analgesics and anti-inflammatories provide pain relief by reducing inflammation but do not alter the disease process. Administer glucocorticoids as ordered. Glucocorticoids decrease pain by suppressing the inflammatory process and may alter the course of the disease. Administer DMARD therapy as ordered. DMARD therapy is very important in slowing disease progression by modulating the immune system and decreasing the inflammatory process. Administer biological therapies as ordered. Biological therapies have been shown to slow or halt the progression of RA through inhibition of tumor necrosis factor (TNF) or interleukins (ILs). Teaching Importance of adherence to treatment plan It is essential to maintain the treatment plan to control disease progression. Report signs and symptoms of infection. Because of treatment with anti-inflammatories and immunosuppressive therapy, patients must be aware of signs and symptoms of infection and the importance of reporting these to their rheumatologist. Immunosuppressive therapy should be discontinued while patients have an active infection. Immunosuppressive therapy puts patients at a higher risk for developing infections and complications from infections. Assist with referral to an infectious disease specialist for patients with chronic or atypical infections. Patients with rheumatoid arthritis who are on immunosuppressive therapy are at risk for developing infections. Keep current with vaccinations. Patients with rheumatoid arthritis are at risk for infections due to treatment but should not receive live vaccines if on immunosuppressive therapy. Refer to physical and occupational therapies as needed. Therapy may preserve joint function and improve patients' quality of life. Evaluating Care Outcomes Important assessments of the efficacy of pharmacological therapy are the amount of joint tenderness and synovitis the patient is experiencing and the duration of morning stiffness. It is possible to achieve remission by using effective medications. Having active patient involvement aids in that process. Teaching the importance of using antirheumatic medications properly and consistently is key in treating RA patients. Patients should understand that full efficacy cannot be achieved if the medication is not used consistently and that the medications can be dangerous if used more than directed. Ideally, the patient with well-controlled RA will have very few or no tender or swollen joints with minimal morning stiffness while experiencing little or no adverse effects from pharmacological therapy. SCLERODERMA Epidemiology The incidence of scleroderma globally is estimated to be 8 to 56 cases per million per year. The usual age of onset is 30 to 50 years; however, the age of onset for female individuals ranges from 15 to 40 years, and the risk for developing scleroderma declines after menopause. The prevalence of scleroderma in the female population exceeds the prevalence in the male population by a ratio of 3:1 to 8:1. The disease affects all races, but Black populations are affected at a higher rate than White populations. Black individuals tend to develop the disease earlier in life and are likely to have more diffuse disease with a worse prognosis. Scleroderma is not considered a hereditary disease, but patients who have a first-degree relative with scleroderma are more susceptible to developing the disease than the general population. It has also been suggested that, as in other autoimmune connective tissue diseases, environmental exposures such as infectious agents and occupational toxins may play a role in triggering the disease. Pathophysiology Scleroderma is a very complex disease that not only affects the skin but also may involve internal organs. The exact cause of scleroderma is unknown, but it is believed to be an autoimmune disease caused by many external triggers. Small-vessel vasculopathy (disease affecting blood vessels), pathological accumulation of collagen, and autoimmunity cause the disease manifestations. Tissue damage occurs when mononuclear cells cluster in tissues such as the skin, blood vessels, and organs, stimulating lymphokines that stimulate procollagen, causing insoluble collagen formation in excess. The inflammatory response causes edema, which leads to fibrotic changes, causing loss of elasticity and movement. Eventually, the tissue degenerates and becomes nonfunctional. Clinical Manifestations Scleroderma is divided into two main categories: localized and systemic. Localized scleroderma leads to morphea (isolated patches of hardened skin), linear scleroderma (lines of thickened skin affecting the tissues beneath), and scleroderma en coup de saber (thickened skin on the head). Localized scleroderma typically does not affect the internal organs. Systemic sclerosis is divided into the categories of diffuse and limited (Table 20.4). Clinical manifestations of diffuse or limited systemic sclerosis may involve the skin, lungs, heart, kidneys, GI, and musculoskeletal systems. Skin manifestations include patches of thickened skin sometimes accompanied by itching, especially in the early stages. Patients with pulmonary involvement may experience dyspnea, nonproductive cough, and pulmonary fibrosis and have an increased risk of lung cancer. Conditions related to cardiac involvement include pericarditis, pericardial effusion, myocardial fibrosis, heart failure, and arrhythmia. Proteinuria and impaired renal function are common with renal involvement. Common symptoms of GI involvement may include dysphagia, heartburn, hoarseness, bloating, and alternating constipation and diarrhea. Musculoskeletal complaints include swelling of the hands, joint pain, muscle pain, and fatigue. Three important manifestations of systemic sclerosis are Raynaud's phenomenon, scleroderma renal crisis, and pulmonary artery hypertension, all of which are due to abnormal vasoconstriction of small vessels as a result of functional changes in the arteries. Raynaud's phenomenon results from vasospasm of the small vessels in the hands caused by exposure to cold. Vasospasm of the small vessels causes the fingers to blanch white, become blue from cyanosis, and then become red from reactive hyperemia. Raynaud's phenomenon is secondary to scleroderma and should not be confused with primary Raynaud's disease. Primary Raynaud's disease is common in the general population and does not result in permanent tissue damage, whereas Raynaud's phenomenon frequently causes permanent tissue damage and loss of digits due to chronic ischemia. Although mild renal dysfunction is common in scleroderma patients, scleroderma renal crisis occurs in approximately 10% to 20% of patients with diffuse systemic scleroderma but rarely in those with the limited cutaneous form. Scleroderma renal crisis is characterized by a sudden onset of severe hypertension, proteinuria, and progressive renal failure. Even with aggressive treatment, morbidity and mortality are high. Pulmonary artery hypertension symptoms develop insidiously, usually with a feeling of generalized weakness on exertion, then later dyspnea. Pulmonary disease is the leading cause of death in patients with systemic scleroderma. See Table 20.5 for clinical manifestations according to organ system. Interprofessional Management Medical Management Diagnosis The diagnosis of scleroderma is supported by the presence of clinical manifestations and serum antibodies. Laboratory testing may confirm a diagnosis of scleroderma but is not beneficial in excluding the diagnosis. Common laboratory tests include antinuclear antibody (ANA) screening, then testing for specific antibodies such as antitopoisomerase I (anti-scl-70), anticentromere, anti-RNA polymerase III, and anti-beta2-glycoprotein I. More than 95% of scleroderma patients have at least one of these antibodies present. Radiographical testing may reveal subcutaneous calcification, distal esophageal hypomotility, and pulmonary fibrosis. Pulmonary function tests are important to assess the vital lung capacity and lung compliance. Echocardiograms should be done routinely to assess for pulmonary artery hypertension and myocardial involvement. Before treatment is initiated, more invasive tests such as bronchoalveolar lavage and right heart catheterization may be required for a definitive diagnosis of interstitial lung disease and pulmonary hypertension, respectively. Kidney biopsies are required to accurately diagnose renal disease. Treatment There is no single treatment to manage scleroderma. Treatment is focused on specific organ involvement and clinical manifestations. Systemic steroids such as prednisone suppress the immune response by reducing the migration of leukocytes and reducing the activity of the lymphatic system. Immunosuppressants, such as methotrexate, work by modulating the immune response to decrease inflammation. Antihistamines, such as loratadine (Claritin) and cetirizine (Zyrtec), may alleviate pruritus by blocking H1 receptors on effector cells. Topical ointments and moisturizers are used to help soften the skin and relieve itching. Vasodilators such as amlodipine (Norvasc) or nifedipine (Procardia) are used to improve circulation and treat Raynaud's phenomenon, pulmonary hypertension, and renal impairment. See Table 20.6 for an overview of treatment options. Complications There are many possible complications related to systemic diffuse scleroderma, including infection, renal failure, heart failure, pulmonary fibrosis, and death. Complications should be addressed by healthcare providers based on the system affected and the degree of involvement. Nursing Management Assessment and Analysis Clinical manifestations of scleroderma are related to multiple factors according to the extent of organ involvement as well as side effects of pharmacological therapy. The nurse may identify the following: Fatigue Thickened or hardened skin Digital ulcers Dyspnea Hypertension Decreased range of motion of joints Poor nutrition Frequent heartburn Dysphagia Cough Nursing Diagnoses/Problem List Altered tissue perfusion related to chronic ischemia Fatigue related to the disease process Altered self-image related to disfiguring skin lesions Impaired movement Pain Nursing Interventions Assessments Vital signs at every assessment: blood pressure, heart rate and rhythm, respiratory rate Elevated blood pressure may be a sign of renal crisis. Elevated heart rate and ventricular arrhythmias may be present due to heart failure. Increased respiratory rate and effort may indicate interstitial lung disease, pulmonary vascular disease, or pulmonary artery hypertension. Auscultate lung sounds. The presence of crackles may be indicative of interstitial lung disease. Assess skin integrity and document skin thickening. Maintaining adequate records of skin involvement is important in monitoring disease progression. Assess the musculoskeletal system. Swelling of the hands, joint pain, muscle pain, and fatigue are common symptoms of scleroderma. Fibrosis around tendons and joint structures may lead to joint immobility. Auscultate bowel sounds and gather information regarding gastroesophageal motility. Chronic gastric reflux, difficulty swallowing, choking, cough, bloating, and alternating constipation and diarrhea are manifestations of GI involvement. Monitor serum creatinine and blood urea nitrogen (BUN). Monitor routinely to assess renal function and possible renal failure. Actions Administer medications as ordered: steroids, immunosuppressants, antihistamines, vasodilators, steroids, topical ointment. Steroids reduce the inflammatory response; immunosuppressants modulate the immune response to decrease inflammation; antihistamines alleviate pruritus by blocking H1 receptors on effector cells; vasodilators dilate arterial vessels, improving circulation; and ointments soften skin and relieve itching. Perform range-of-motion exercises. Range-of-motion exercises improve joint mobility and help prevent contractures. Assist in referring the patient to pulmonologist, gastroenterologist, plastic surgeons, and physical therapy as needed. Diffuse scleroderma may lead to decreased pulmonary function, GI dysfunction, disfiguring skin lesions, and decreased joint mobility. Refer patients to counseling as needed. Many patients suffer from depression as a result of the diagnosis and clinical manifestations. Teaching Scleroderma disease process. Scleroderma is a very complicated disease that varies from one patient to another. Patients should understand and monitor for possible complications but realize that they may not experience all possible complications. Protect skin from trauma. Patients are at higher risk for infection and prolonged healing time related to decreased tissue perfusion. LUPUS Epidemiology Systemic lupus erythematosus (SLE) affects fewer than 25 people per 100,000 in North America, South America, Europe, and Asia. Prevalence rates vary depending on sex, ethnicity, geographical location, and age. The female-to-male ratio in children affected by SLE is 3:1 but is as high as 15:1 for female individuals during childbearing years. Statistically, African American female individuals are almost four times more likely to develop the disease than European American individuals; however, SLE occurs infrequently in Black populations in Africa, Asians, and African Caribbeans. Hispanic Americans have a higher prevalence rate when compared to European Americans in the United States. The age of onset varies according to gender and ethnicity, but overall, 65% of patients are diagnosed between the ages of 16 and 55. Many factors play a role in disease outcome. African Americans and Mexican Americans have a poorer prognosis related to renal disease than do European Americans. Less-educated patients and those with low socioeconomic status are also more likely to have poorer disease control. Males are more likely than females to have renal disease, skin manifestations, laboratory abnormalities, neurological involvement, thrombosis, cardiovascular disease, and vasculitis, whereas females are more likely to suffer from Raynaud's phenomenon, photosensitivity, and mucosal ulceration. People who have relatives with SLE are at a higher risk than the general population for developing the disease. There have been genes identified that predispose humans to SLE. Pathophysiology Systemic lupus erythematosus is a chronic inflammatory disease that can affect virtually any organ system. The exact etiology is unknown, as is the case with other autoimmune diseases, but the disease is triggered by multiple factors. Triggering factors include pregnancy, exposure to sunlight, illness, major surgery, silica dust, and medication allergies. Most clinical manifestations of SLE are attributed to antibodies and the creation of immune complexes that are deposited into tissues. Clinical Manifestations Clinical manifestations of SLE are diverse, vague, do not follow a clinical pattern, and vary greatly from one person to another. They can include symptoms such as fatigue, fever, myalgia, and weight changes. Symptoms are not always present in every patient, increasing the difficulty of diagnosis. The clinical course of SLE usually involves periods of remission and acute disease flares. See Table 20.7 for a description of clinical manifestations according to organ system. Interprofessional Management Medical Management Diagnosis Diagnosing lupus may be a complicated and frustrating process for most patients. There is no specific test used to diagnose SLE; rather, laboratory findings are used to support or confirm the diagnosis when combined with patient history and physical examination findings. The ACR developed guidelines to help in the diagnosis of lupus. The Systemic Lupus International Collaborating Clinics revised and validated the classification criteria to aid in diagnosis. They identified 17 broad criteria that align with the clinical manifestations with specific definitions---some clinical, some immunological. The clinical criteria include skin rashes such as the lupus malar rash, oral ulcers, thinning hair, joint tenderness, pleural or pericardial effusions, renal (urine protein) and neurological (seizures/confusion) disorders, and hematological disorders such as thrombocytopenia, leukopenia, or anemia. The immunological criteria include positive ANA, low complement, positive anti-Sm, antiphospholipid antibodies, and positive anti--double-stranded DNA (anti-dsDNA). Four of the 17 criteria must be present to support a diagnosis of SLE---at least one clinical and one immunological. In clinical practice, however, patients may not have four criteria present to be diagnosed if there is sufficient evidence otherwise. Laboratory Testing Laboratory testing is used to confirm some of the clinical and immunological criteria. First, testing is done to confirm the presence of autoantibodies. Patients with SLE produce ANA, confirming the existence of an autoimmune disease. The presence of ANAs does not in itself confirm the diagnosis of SLE because approximately 2% of healthy individuals are positive. Anti-dsDNA and anti-Sm antibodies are most specific for SLE because these autoantibodies are rarely found in other conditions. Antiphospholipid antibodies should also be assessed because the presence of these antibodies may lead to the formation of blood clots. Laboratory values to evaluate the clinical criteria include a complete blood count (CBC) to check for leukopenia (decreased white blood cell count), thrombocytopenia (decreased platelet count), and anemia (decreased red blood cells). Urinalysis with random protein and creatinine along with serum creatinine and BUN should be evaluated and monitored closely in order to detect kidney disease early. C-reactive protein and ESR are markers used to identify inflammation but are not specific for SLE. C-reactive protein and ESR may be monitored to assess for level of disease activity and response to treatment. Radiographical Imaging Radiographical imaging is beneficial to detect or assess some of the clinical criteria or to eliminate other causes of symptoms. See Table 20.8 for common radiographical diagnostic tools used to assess patients with SLE. Treatment Nonpharmacological Therapy Nonpharmacological interventions include avoiding prolonged sun exposure and using sunscreen (SPF 50 or higher) on a daily basis. A well-balanced diet is recommended for proper nutrition. Frequent rest periods and a regular sleep schedule help combat fatigue. Regular exercise improves strength and maintains range of motion, as well as a healthy weight. Patients who are smokers should be encouraged to quit because smoking has been associated with more active disease. Pharmacological Therapy Pharmacological interventions for patients with SLE are typically based on disease manifestations. Antimalarial medications such as hydroxychloroquine (Plaquenil) are frequently used to treat patients with SLE. The exact mechanism of action of hydroxychloroquine is not fully understood; however, it is believed to impair complement-dependent antigen--antibody reactions. Hydroxychloroquine is also useful in treating constitutional symptoms such as fatigue, as well as skin and joint manifestations. It also helps to prevent disease flares and serious organ disease such as lupus nephritis. The most common side effects related to hydroxychloroquine use are abdominal pain and nausea, which may improve over time Nonsteroidal anti-inflammatory medications such as ibuprofen are useful in treating arthralgias, myalgias, headaches, and fever. Glucocorticoids may be necessary to suppress inflammation in joints, kidneys, and other organ systems. Immunosuppressive agents such as methotrexate may be necessary to treat joint inflammation, which does not respond to NSAIDs or steroids. Glucocorticoids and immunosuppressive agents may be used in combination with hydroxychloroquine in order to treat more serious clinical manifestations. Belimumab (Benlysta) is a biological response modifier that was approved in 2011 for the treatment of SLE. It is the first new treatment to be approved for the treatment of SLE in more than 40 years. Belimumab is made of monoclonal antibodies that bind to proteins known as BLyS and interferes with the inflammatory cascade, decreasing the immune response that is responsible for causing clinical manifestations of SLE. When using belimumab or other biological agents to treat SLE be aware that significant drug interactions exist. Nurses and patients should make sure that drug lists are up to date, including OTC medications. Surgical Management Surgical interventions may include renal transplant for patients with severe lupus nephritis and joint replacement for patients at risk for avascular necrosis of large joints. Complications Lupus is a chronic disease with many possible complications, including renal failure, premature heart disease, interstitial lung disease, hypercoagulation, stroke, avascular necrosis of joints, and increased risk for infection. Complications may also arise from toxicities related to pharmacological therapy. Nursing Management Assessment and Analysis There are many clinical manifestations related to SLE. The etiology is unknown, but it is thought to be attributed to antibodies and the creation of immune complexes, which are deposited into tissues. The nurse may identify the following: Fatigue Difficulty concentrating Joint pain Rash Photosensitivity Oral or nasal ulcers Dry eyes Dry mouth Hypertension Leukopenia Thrombocytopenia Alopecia Chest pain Nursing Diagnoses/Problem List Fatigue related to chronic inflammation and altered immunity Altered skin integrity related to rash Altered self-image related to manifestations such as rash and alopecia Nursing Interventions Assessments Vital signs Hypertension may occur as a renal or cardiac complication of SLE, fever may be present due to infection that is a complication of treatment with immunosuppressants, and decreased oxygen saturation may be present because of the complication of interstitial lung disease. Past health history/head-to-toe physical assessment The diagnosis of SLE is based on the presence of 4 of 17 clinical manifestations in the patient's history and physical assessment, such as butterfly rash, oral or nasal ulcers, alopecia, musculoskeletal complaints, joint swelling, weight loss, and blood abnormalities. Monitor laboratory values: BUN and creatinine, urinalysis, CBC, CRP/ESR, coagulation studies. Elevated BUN and creatinine along with the presence of proteinuria may indicate decreased renal function and is one of the clinical criteria for SLE. Decreased white blood cells, platelets, and red blood cells are common in patients with SLE and may require intervention. Elevated CRP and/or ESR indicates inflammation and may require intervention. Coagulopathies are a complication of SLE. Actions Administer analgesics and anti-inflammatory medications as ordered. Most patients require analgesics and anti-inflammatory medications to manage the pain caused by inflammation. Administer medications as ordered to treat specific clinical manifestations. Managing clinical manifestations is imperative in the treatment of patients with SLE to help prevent complications. See Table 20.9 for medications used to treat clinical manifestations. Teaching Disease process It is very important for patients to understand the disease process in order to manage the disease and help prevent complications. Use sunscreen daily. Photosensitivity is common. Daily use of sunscreen helps prevent rash related to photosensitivity. Energy conservation and activity prioritization Fatigue is a common complaint and can be better managed by ensuring frequent rest periods and prioritizing activities. Keep up to date on immunizations, but avoid live vaccines. Patients at risk for infections due to treatment should not receive live vaccines if on immunosuppressive therapy. Avoid oral contraceptives in patients with SLE who have migraine headaches, Raynaud's phenomenon, a history of phlebitis, or antiphospholipid antibodies. Patients with SLE, specifically patients with the manifestations noted, have an increased risk of hypercoagulability, which may be heightened by the use of oral contraceptives. Assist in referrals to a pulmonologist, nephrologist, neurologist, cardiologist, and dermatologist as needed. Complicated clinical manifestations require the care of specialists to provide optimal care. Evaluating Care Outcomes Systemic lupus erythematosus is a complicated disease that can be very difficult to manage. Medication compliance plays a major role in controlling the disease. Disease flares are not unexpected and may consist of increased fatigue, fever, rash, arthritis, and mucosal ulcers, as well as other common clinical manifestations. Frequent evaluations should be performed on a regular basis by a rheumatologist who monitors disease progression and treats new clinical manifestations as they arise. Patients with well-controlled SLE are high-functioning individuals who are able to carry out the usual activities of daily living. GOUT Epidemiology Gout affects approximately 2% of people in the United States and is most commonly found in males ages 40 to 60 years and females ages 55 to 70. It is rare to find gout in children. Risk factors for developing gout include obesity, hypertension, eating large amounts of meat and seafood, using thiazide diuretics, and consuming large quantities of alcohol. When metabolized, purine-rich foods such as seafood, meat, and alcohol increase uric acid production. Thiazide diuretics, such as hydrochlorothiazide, increase the net urate reabsorption in the renal tubules, therefore increasing the serum uric acid level. Pathophysiology and Clinical Manifestations Gout is a disease in which monosodium urate crystals are deposited in joints, bone, and soft tissues, accompanied by inflammation (Fig. 20.7). Hyperuricemia, an elevated uric acid level in the blood, must be present before the evolution of gout, but the presence of hyperuricemia is not indicative of gout. Hyperuricemia results from the overproduction of or diminished renal excretion of uric acid. Urate crystals are the result of an oversaturation of uric acid in the blood. The uric acid settles out of solution into soft tissues and joints. The body's attempt to rid itself of the crystal deposits results in an inflammatory process causing swelling, warmth, and intense pain in the affected joint. Gout attacks typically resolve after several days or weeks even if left untreated; however, gout can become a chronic inflammatory destructive arthritis. There are three phases of gout, which are termed acute, intercritical, and chronic. Acute gout usually involves one joint and is characterized by acute onset of pain, redness, and swelling. Intercritical gout is the asymptomatic period between gout attacks. If gout is left untreated, the attacks may come more often, with a shortened intercritical period. Chronic tophaceous gout is characterized by repeated attacks of many years, leading to the production of tophi (uric acid deposits or nodules in the joint) and joint destruction. About 80% of patients present with the first attack of gout affecting a lower extremity joint, typically the first metatarsophalangeal (MTP) joint. Podagra is the term used to identify gout in the first MTP. The severity of the attack typically peaks at 12 to 24 hours after onset. Patients may experience gout flares that are provoked by a variety of dietary and physical factors. These factors include trauma, surgery, fatty foods, use of drugs that affect serum urate concentrations (such as diuretics), and alcohol consumption. Triggers for gout flares may also include immunizations. Gout flares are typically monoarticular and occur in the lower extremities. Interprofessional Management Medical Management Diagnosis A presumptive diagnosis of gout may be achieved by combining objective, subjective, and laboratory data, but the definitive diagnosis can be made only by observing crystals in synovial fluid or tophaceous material. Radiographical evidence is not necessary to diagnose gout; however, joint erosions and soft tissue nodules are radiographically consistent with chronic gouty arthritis. See Table 20.10 for the typical presentation of gout patients. Other diagnoses such as septic arthritis, trauma, and pseudogout should be ruled out. The presentation of septic arthritis and pseudogout may mimic acute gout and may not be differentiated by clinical examination alone. In some cases, it is necessary to examine joint fluid for uric acid crystals to make a definitive gout diagnosis. Treatment Nonpharmacological Therapy Nonpharmacological treatment consists of weight management because obesity is a modifiable risk factor for gout. Alcoholic beverages, specifically beer, should be avoided because they increase uric acid production. Splinting of the affected joint may also be useful in protecting the joint and alleviating pain. Pharmacological Therapy Pharmacological treatment of gout depends on which stage the patient is in when requesting treatment. Acute gout management is focused on pain relief and reduction of inflammation. Common medications used include NSAIDs such as indomethacin (Indocin) and colchicine (Colcrys), which decrease the buildup of uric acid crystals in the joint. Glucocorticoids are used to reduce inflammation and provide pain relief especially in those patients who have contraindications to NSAID use. Management of intercritical gout consists of the use of NSAIDs and colchicine, whereas managing chronic gout focuses on the use of uric acid--lowering agents. Uric acid--lowering agents such as allopurinol (Zyloprim) or febuxostat (Uloric) reduce serum uric acid by inhibiting the production of uric acid. One very important fact to remember is that acute gout is never treated with uric acid--lowering agents such as allopurinol. For unknown reasons, a rapid change in serum uric acid levels may provoke or worsen a gout attack. Complications Urate crystals can aggregate to form a stone, making gout patients more likely to experience kidney stones than the general population. Nursing Management Assessment and Analysis Clinical manifestations of gout are related to pain and decreased function due to the accumulation of urate crystals and tophi nodules in the affected joints. The nurse may identify the following: Intense joint pain Tenderness on palpation of affected joint Swelling of affected joint Redness of affected joint Warmth over affected joint Decreased range of motion Presence of tophi Nursing Diagnoses/Problem list Pain related to the disease process Knowledge deficit related to the disease process and treatment Nursing Interventions Assessments Monitor uric acid level. Hyperuricemia promotes crystal formation. Assess for presence of tophi---lumps or hard nodules under the skin around joints. The presence of tophi is indicative of advanced gout. Assess for red, swollen, and painful joints. Red, swollen, and painful joints indicate an acute gout flare. Assess pain levels. Evaluating pain helps determine the occurrence of a flare and also evaluates the efficacy of treatment. Actions Administer uric acid--lowering agents as directed. Uric acid--lowering agents are necessary to decrease serum uric acid and decrease the incidence of gout flares in chronic gout. Administer analgesics and anti-inflammatory medications as ordered. Anti-inflammatory agents and analgesics must be used to help control pain and decrease inflammation in acute or intercritical gout. Administer glucocorticoid therapy as directed. Systemic glucocorticoid therapy may be used in conjunction with or as an alternative to NSAID therapy to reduce inflammation in acute gout. Teaching Avoid alcoholic beverages, especially beer. Alcohol is high in purines, which metabolize into uric acid, increasing serum uric acid levels. Take uric acid--lowering agents as directed. The use of uric acid--lowering agents is necessary to decrease serum uric acid levels and help prevent future attacks. Report gout flares promptly. Prompt treatment decreases the pain and the severity of gout flares. Proper nutrition Avoid high-purine foods, such as red meat, liver, and fish. FIBROMYALGIA Epidemiology Fibromyalgia (FM) is the most common cause of chronic musculoskeletal pain in female individuals between the ages of 20 and 55. The disorder affects approximately 4% of the U.S. population, with a greater prevalence in the female population. Fibromyalgia is commonly found in patients with rheumatic conditions such as RA, SLE, and Sjögren's syndrome. The onset of fibromyalgia usually occurs in middle adulthood but may occur in adolescence or later in life. Genetic factors may play a role in predisposing patients to the development of FM. Pathophysiology and Clinical Manifestations Fibromyalgia is a chronic pain disorder of soft connective tissues that is characterized by widespread pain and other symptoms such as insomnia, fatigue, stiffness, and cognitive dysfunction (e.g., inability to focus or concentrate on tasks). The etiology and pathophysiology of this disorder are unclear, but current theories include the abnormal processing of stimuli by the central nervous system, causing normal pain signals to be amplified. The amplification of stimuli causes FM patients to feel pain, whereas those without FM would not feel pain. Fibromyalgia may be triggered by an infection or physical or emotional trauma. In females, the menstrual cycle can affect sensitivity to pain. Normal daily stress combined with common FM symptoms can create a cycle that builds one on another (Fig. 20.8). Interprofessional Management Medical Management Diagnosis Laboratory testing is not necessary to diagnose FM and should be limited to those tests necessary to rule out other diagnoses. History and physical assessment are used to diagnose FM. Patients often report hurting all over or feeling as if they have the flu. Other complaints include fatigue, headaches, cognitive impairments, and abdominal pain. Physical examination of the patient is essentially normal, with no evidence of joint or muscle inflammation. The 2010 ACR preliminary diagnostic criteria for FM do not require a tender point examination and provided a scale for measurement of the severity of symptoms that are characteristic of FM. These criteria were based on patient self-reporting, making a complete history a critical part of the examination. According to the 2012 criteria a patient satisfies FM criteria if the following three conditions are met: (1) widespread pain index greater than 7; (2) symptoms that have been present for at least 3 months; and (3) there is no other disorder that would explain the patient's symptoms Treatment Nonpharmacological Therapy Nonpharmacological therapy includes physical therapy, strength training, aerobic exercise, cognitive-behavioral therapy, education, and self-management. Physical therapy is helpful in maintaining strength and function. Cognitive-behavioral therapy may be used to help understand the illness and change patients' behavioral response to pain. Self-management skills include utilizing relaxation techniques to minimize stress, establishing regular sleep patterns, and exercising regularly. Pharmacological Therapy Pharmacological therapy includes medications that alter chemicals in the brain, such as serotonin and norepinephrine, or block overactivity of nerve cells. Other medications that may prove useful are those that improve sleep and treat pain. The use of opioid analgesics is not recommended because they may be habit forming and usually are ineffective. Table 20.11 provides an overview of medications used for the treatment of FM. Nursing Management Assessment and Analysis The nurse may identify the following clinical manifestations in FM patients due to enhanced pain perception and stress related to that enhanced pain: Fatigue Arthralgia and myalgia Headache Abdominal pain Decreased desire to participate in activity Nonrestorative sleep Anxiety Nursing Diagnoses/Problem List Chronic pain related to the disease process Ineffective coping skills related to chronic pain Depressed mood related to chronic pain Ineffective sleep patterns related to chronic pain Nursing Interventions Assessments Vital signs Pain may alter vital signs (increased heart rate and blood pressure). Affect Depression may be an associated symptom because of chronic pain. Pain Fibromyalgia causes chronic pain. Increased levels indicate ineffective treatment strategies, whereas decreased pain levels indicate effective treatment and self-management Actions Administer medications as ordered: Nonsteroidal anti-inflammatories Antidepressants Antiseizure medications Pain management is an integral part of managing patients with FM. Nonsteroidal anti-inflammatories manage inflammation. Antidepressants manage depression, promote sleep, and help control pain through inhibition of the reuptake of neurotransmitters, which inhibit pain transmission in the brain. Antiseizure medications work for unknown reasons but may modify the release of neurotransmitters through action on calcium channels. Provide a heating pad for painful muscles and cold packs for painful joints. Heat is used to relax muscles, increasing blood flow to the affected area; cold packs may soothe aching joints. Teaching Take medications only as prescribed. Improper use of medications can be very harmful. Proper use of medications is required in order to receive the desired effect. Participate in regular physical activity. Physical activity is necessary to maintain or improve strength and function. Teach effective coping skills. Effective coping skills are important to maintain a positive attitude and improve self-concept. Assist with referring the patient for a sleep study. Ineffective sleep may increase pain and fatigue. Assist with referring the patient to a mental health facility if necessary. Fibromyalgia patients may benefit from therapy to help deal with depression related to chronic pain. Assist with referral to occupational and physical therapies as needed. Occupational and physical therapists may formulate an exercise plan appropriate for the patient. Evaluating Care Outcomes Compliance with a healthy diet and exercise regimen and getting adequate rest are essential in managing FM. Taking medications as prescribed helps to control the enhanced sensation of pain and the depression associated with the disease process. A well-managed patient has decreased pain and fatigue, healthy sleep patterns, and maintenance or improvement of strength and function