Rheumatology Lecture Notes PDF

[Rheumatology Lecture Notes ] Seronegative Spondyloarthropathies (SPA) - Spondylarthritis is a term used for this family of disorders (many clinical features are similar across these disorders) - Disorders = PEAR - P - Psoriatic Arthritis - E - Enteropathic Spondyloarthropat...

[Rheumatology Lecture Notes ] Seronegative Spondyloarthropathies (SPA) - Spondylarthritis is a term used for this family of disorders (many clinical features are similar across these disorders) - Disorders = PEAR - P - Psoriatic Arthritis - E - Enteropathic Spondyloarthropathies - A - Ankylosing Spondylitis - R- Reactive Arthritis - Clinical features of SPA - **Rheumatoid Factor Negative** - Inflammatory back pain (\>3 months) - Sacroiliitis - Inflammation of bone insertions of ligaments and tendons - Peripheral joint inflammation - Often eye inflammation and skin disease (psoriasis associated with all forms of SPA) - May have inflammation of bowel mucosa - **Ankylosing Spondylitis (AS)** - Most common seronegative spondyloarthropathy - Begins in 3^rd^/4^th^ decade (usually in 20s), rarely beyond age 45 - Chronic systemic inflammatory disease impacting - Sacroiliac joints - Intervertebral disc spaces - Peripheral joints - Primary vs secondary - Primary = no associated disorder present - Secondary = associated reactive arthritis, psoriasis, UC, Chrons - **Patients have chronic back pain and progressive spinal stiffness -- disabling** - Patho - Genetic and environmental - HLA-B27 most common predisposing gene - Inflammation of joints and adjacent tissue cause formation of granulation tissue leading to joint fusions - Pathologic features consistent with bone erosion, remodeling, and new poorly formed bone formation - Inflammation can impact the eyes, lungs, heart, kidneys and peripheral nervous system - **Inflammation \> Granulation tissue \> Erosion \> Fibrocartilage \> Ossification of the joint** - Clinical Presentation - Low back pain and or stiffness - Spondylitis begins in the sacroiliac joints - As disease progresses, the upper portions of the spine become involved - Back pain = inflammatory - Pain \> 3 months - Insidious onset - SI Joint involvement is common - Morning stiffness lasting \> 1 hour - Pain increases with inactivity and improves w/ movement - Extra-articular Manifestations of AS - May have constitutional symptoms (low grade fever/fatigue) - Weight loss - Bowel -- inflammatory bowel disease - Skin -- psoriasis - Cardiac -- increased risk of atherosclerosis - Eye -- unilateral ocular redness, pain, blurred vision - Pulmonary -- pulmonary fibrosis or interstitial lung disease - Physical Exam for AS - Exam of spine - Loss of normal lumbar lordosis - Muscle spasms frequently present - Spine mobility decrease in all planes (most in lumbar/cervical) - Chest expansion - Measured w/ pt standing erect w/ hands on head - Tape measure wrapped around the chest at the nipple line - Have pt max inspire and expire (normal = increase by at least 5 cm) - Becomes limited w/ progression of disease due to thoracervical kyphosis - SI joint tenderness - Tenderness at insertion sites - Eval for extra-articular manifestations - Diagnostics for AS - Lab findings generally nonspecific (CBC/CMP) - Inflammatory markers -- ESR/CRP may be normal or mild elevation - HLA-B27 testing - RF and ANA normally negative as this is SERONEGATIVE disease - Dx made w/ symptoms and presence of HLA-B27 - **Radiology -- Key to diagnosis** - **Plain films of pelvis, lumbar spine and Cspine for all pt suspected of AS** - Presence of sacroiliitis on xray is definite AS - **Syndesmophyte (bony growth originating from ligament) = bamboo spine appearance is characteristic finding** - Management of AS - Goals of Tx - Relieve symptoms - Maintain best functional capacity possible - Prevent complication of disease - Minimize extra-articular manifestations - Achievement of goals depends on - Pt education - Exercise - Medication management - **NSAIDS ARE FIRST LINE THERAPY** - Biologic anti tumor necrosis factor (TNF) and disease modifying anti rhematic drugs - Use of systemic glucocorticoids is NOT recommended for long term use - Pain management is crucial to allow for exercise - Refer - Rheumatology = formal dx, management and monitoring - Spinal surgeon -- management of complications as needed - GI -- bowel - Derm -- Psoriasis - Optho -- uveitis **Reactive Arthritis (aka Reiter Syndrome)** - Arthritis that develops soon after or during infection elsewhere in body - Source - Usually GI/GU source - Chlamydia, trachomatis, salmonella, shigella, campylobacter, E coli, Cdiff - Peak incidence = young adult (3^rd^ decade of life) - Post infectious triad -- arthritis, urethritis, conjunctivitis - "Can't see, can't pee, can't bend my knee" - 2 forms of disease - Sexually transmitted = 7-14 days post exposure to chlamydia - Posenteric infection -- usually includes travelers diarrhea - Clinical Presentation of reactive arthritis - With infection -- arthritic symptoms occur within 1-4 weeks - Classic Triad - Eye involvement -- blepharitis, conjunctivitis - Arthropathy -- asymmetric, usually large joints of lower extremities - Spine is less involved - Urethritis -- may be transient - MOST SYMPTOMS RESOVE WITHIN 1 year - Extra-articular Manifestations - Constitutional - Malaise, fever, fatigue, HA, weight gain - May appear quite ill - Dermatologic - Painless, shallow, lingual or palatal ulcerations - Circinate balanitis -- seen in males - Painless, asymptomatic, shallow, ulcerative lesions of glans of penis - Erythema nodosum - Keratoderma blennorrhagia - Most common derm manifestation - Appears as painless papulosquamous lesions on palms or soles - Hyperkeratotic skin - May be mistaken for psoriasis - Diagnostics for reactive arthritis - ESR/CRP -- will be elevated - CBC -- leukocytosis, thrombocytosis, monochromic anemia - RF -- NEGATIVE (seronegative disorder) - Imaging -- none - Synovial fluid -- nonspecific - Infection -- stool culture, UA, genital swab (chlamydia testing) - Ddx for reactive arthritis - Difficult to determine psoriatic arthritis and reactive arthritis (arthritic symptoms are similar and skin histology is identical) - Lyme disease - Seronegative RA - Gonococcal arthritis - Gout - Septic arthritis - Inflammatory bowel disease - Management of reactive arthritis - Tx underlying infection (may need abx) - NSAIDS as initial tx - Intraarticular glucocorticoids -- those that done respond to NSAIDS - Systemic glucocorticoids - Use if not responding to NSAIDS - Contraindications to NSAIDS (GI bleed, peptic ulcer, renal disorder) - Low dose prednisone 20 mg **Psoriatic Arthritis** - Chronic inflammatory arthritis associated with dermatologic condition of psoriasis - Clinical manifestations - Worse in AM and with prolonged immobility - Joint manifestations - Digital arthritis (DIP joint involvement) - Asymmetric oligoarthritis - Symmetric polyarthritis - Arthritis mutans - Spondylarthritis - Periarticular disease -- tenosynovitis, enthesitis, dactylitis (sausage finger) - Nail lesions -- nail pits, hyperkeratosis, splinter hemorrhages - Edema of hands and feet - Ocular inflammation 0 uveitis and conjunctivitis - Diagnostics - ESR/CRP = Elevated - CBC = normocytic anemia/leukocytosis - RF and ANA = NEGATIVE (seronegative disease) - Hyperuricemia -- may be 2/2 high purine turnover and psoriatic skin lesions - Rad -- changes in hand and feet are distinctive - Dx can be made in pt who has both psoriasis and inflammatory arthritis in a pattern typical of psoriatic arthritis - Management - NSAIDS -- first line therapy - Many pt have continued symptoms despite NSAIDS, require DMARDS second line - Consult rheumatology and dermatology recommended **Polymyalgia Rheumatica and Giant Cell Arteritis (PMR/CGA)** - Polymyalgia Rheumatica (PMR) - Treatable, chronic systemic inflammatory condition of unknown cause - Characterized by - Diffuse aching, stiffness in shoulder girdle, neck, pelvic girdle - Giant cell arteritis (GCA) - Most common type of vasculitis in adults, affects medium and large arteries and can result in blindness - PMR/GCA typically occur together (well recognized to occur together) - Seen in elderly (women \> men) - DOES NOT OCCUR IN PT YOUNGER THAN 50 YR OLD - Dx made on clinical grounds after exclusion of other inflammatory conditions - Patho - Cause = unknown - Genetic/immune/environmental factors believed to be important - Higher incidence during winter months - Higher prevalence in rural areas and urban communities Polymyalgia Rheumatica - Clinical Presentation - Abrupt onset - Involves muscles and joints of shoulder, neck, hip girdle - Usually symmetric - Morning stiffness (ABSENCE EXCLUDES DX OF PMR) - Functional limitations -- can limit ADLS - Systemic s/s - Malaise, fatigue, depression, anorexia, weight loss, LOW GRADE FEVER - Physical Exam - Joint exam - Should ROM Decreased -- classic finding - UNABLE TO ABDUCT SHOULDERS - Unusual manifestation is involvement of puffy, edematous hand syndrome - Neuro exam - Normal muscle strength - Tenderness to msk palpation is nonspecific sign - Diagnostics (No definitive dx test for PMR) - CBC - Normochromic Normocytic anemia - ESR and CRP - CMP - Serum protein electrophoresis - Rheumatoid factor = normal - Anti CCP = Negative - Management - Corticosteroids = standard therapy - Low dose prednisone typically induces a rapid response within a few days - NSAIDS not recommended - Steroids often needed for several years (may stop 6-12 months if symptom free) - Lab abnormalities should improve within 4-8 weeks - Refer -- manage in conjunction with rheumatology Giant Cell Arteritis (GCA) - Clinical presentation = impacts med/large arteries - Classic presentation - Headache -- most common presenting symptom (responds poorly to analgesics and is sensitive to touch/combing) - Fatigue, weight loss, anorexia - Jaw claudication - Jaw claudication and visual symptoms are ischemic warning signs - Diplopia, burning and amaurosis fugax often precede permanent visual loss - Patho - Transmural inflammation involving all layers of arterial wall, intimal hyperplasia leads to occlusion of vessel lumen - Presence of mononuclear infiltrates or granulomas with multinucleated giant cells - Arteries - Carotid artery and branches - Great arteries especially thoracic aorta and branches - Stroke, blindness, other clinical manifestations are caused by tissue ischemia secondary to luminal occlusion - Physical exam - Dx made on constellation of symptoms and lab testing - Palpate temporal arteries - Exam of scalp - Auscultation of neck - BP reeding in both arms - Ocular fundoscopy - Rheumatology classification criteria for Giant Cell Arteritis (KNOW THESE) - Age of onset \> 50 years - New headache - Temporal artery abnormality - Elevated ESR - Abnormal artery biopsy - \*\*\*These are not diagnostic but guides ddx of GCA - Management - Prednisone high dose therapy -- mainstay of treatment - 1mg/kg w/ max daily dose 60 mg/day - Typically for 1 month and tapered slowly - Duration of therapy variable - Average duration 2-3 years - Low dose ASA therapy to prevent ischemic complications (vision loss) - Complications - Relapses common - Risk for MI, stroke, peripheral vascular disease is substantially increased **Raynaud Phenomenon** - Episodic, reversible vasospasm of peripheral arteries - Causes pallor, followed by cyanosis and redness (can develop ulcerations) - Associated with pain and possibly paresthesia - Primary vs secondary - Primary -- no underlying disease - Secondary -- associated autoimmune disorder (scleroderma), more severe and less symmetric - Can also be associated with drugs and trauma - Seen in patient with history of migraine headaches and chest pain - Patho - Cause = unknown - Blood vessels constrict in response to cold or stress - May be related to increased activation of sympathetic nerves - Resultant disturbance in circulation causes series of skin changes - White, blanches or pale as blood flow is reduced - Blue as digit loses oxygen - Red or flushed as blood flow returns - As attach resolves normal blood flow is restored and color returns - May experience numbness and tingling and palpable coldness in first two stages - Red stage -- warmth, burning and swelling may occur - Pain is common complaint (especially in secondary Raynaud's) - Fingers and toes most commonly impacted - Earlobe, nose, rarely tongue can also be impacted - Clinical presentation - Vasospasm causes classic tricolor changes - White \> Blue \> Red - Episodes triggered by cold exposure, rapid changes in ambient temp, emotional stress - Attacks occur in single or multiple digits (generally symmetric) - Can spread to other digits, other hand, or feet - May last between 20 min and hours - Dx made = pallor of one or more digits with color change - Diagnosis - Based on classic hx of tricolor changes - ANA titer -- positivity is predictor of progression association with connective tissue disorder - ESR normal - Primary = ANA negative and SED is normal - Secondary -- positive ANA and look for other causes - Management - Environmental measures = first line - Keep body warm - Use stress management - Avoid smoking - Estrogen, BBlockers, pseudoephedrine should be avoided - Nifedipine - May be helpful in prevention of vasospasm - Vasodilators may be used on occasion - No one tx seems preferable (nifedipine/amlodipide) - AVOID ASA with all pt with secondary Raynaud phenomenon **Rheumatoid Arthritis (RA)** - Autoimmune disorder w/ SYMMETRIC inflammatory polyarthritis - Family hx of RA or other autoimmune is common - Cause = unknown - May have genetic, environmental, hormonal, reproductive factors - Smoking shown association - Insult (such as infection/smoking/trauma precipitates an autoimmune reaction) - Patho - Main target of inflammation is synovial lining of diarthrodial joints - Early changes are seen in capillaries and blood vessels - Synovial membrane undergoes hyperplastic thickening - Subsequently granulation tissue develops called "pannus" - Invades cartilage and subchondral bone - Primary responsible for destruction of joint structures - Overproduction of pro-inflammatory cytokines, including tumor necrosis factor (TNF) and interleukin 6 drives destructive process A diagram of a person\'s body Description automatically generated Notice -- inflammation and presence of B and T cells (normal joint to left, RA joint on right) Clinical presentation of RA - Onset usually insidious - Initial symptoms - General systemic manifestations - Weakness, weight loss, malaise, fatigue, anorexia, aching and stiffness - Localized symptoms include - Painful, tender, swollen joints - Usually asymmetric - Boggy swelling due to synovitis may be present - Morning stiffness lasts minimum of 1 hour (sometimes all AM) - Commonly affects - Small joints of hands - Wrists - Small joints of feet - Typically involves at least three joint groups  RA -- presence of swelling and boggy joints but with OA there is not Specific signs for the hand with RA - Early signs - MCP, PIP, wrist or MTP joint swelling - Later - Boutonniere deformity - Flexion of the PIP and hyperextension of DIP joints - Swan neck deformity - Hyperextension of PIP and flexion of DIP joints - Ulnar deviation - Subluxation of proximal phalanges towards ulnar side  Diagnostic Criteria for RA - Based upon presence of synovitis in at least one joint - Absence of dx that would better explain the synovitis - Achievement of total score of at least 6 (of possible 10) - Number and site of involved joints - Serologic abnormalities (RF or antiCCP) - Elevated ESR/CRP - Symptom duration at least 6 weeks - UpToDate - Inflammatory arthritis of 3 or more joints - \+ Rheumatoid factor - Elevated CRP or ESR levels - Disease similar have been excluded - Duration of symptoms is \> 6 weeks Physical Exam for RA - Examine peripheral joints and axial skeleton - Pain, tenderness, degree of swelling, ROM, deformity - Palpate affected joints - Inflamed joint with warm and tenderness - Synovial membrane may be palpable as thickened and boggy - Evaluation of extra-articular manifestations - Subcutaneous nodules (found over pressure areas) - Ocular signs (Sjogren syndrome and Uveitis) - Respiratory (interstitial lung disease) - Key features - Pain and selling of affected joints - Synovial thickening "boggy sensation" - DIP joints are affected with FLEXION noted - MCP joints frequently involved with RA Diagnostics for RA - Primary based on clinical hx and physical exam - Lab testing and radiographs can confirm dx - Baseline eval - CBC = normocytic, normochromic anemia common - ESR and CRP - CMP - Rheumatoid Factor (RF) - Preferred test for dx, RF alone is not enough to dx, anti CCP antibodies are more specific and may be detected earlier in disease (Anti CCP will be positive) - Anti CCP antibodies - Lipid testing - Annual lipid testing recommended - Pt at increased cardiovascular risk 2/2 inflammatory burden - Should be evaluated annually and repeated w/ antirheumatic treatment changes - Eval renal and hepatic function -- many antirheumatic drugs have renal and hepatic toxicity so need baseline - Xrays - Hand and foot studies -- establish baseline - No evidence in initial phases, beyond 6 months will reveal bony erosions - Synovial fluid - Aspiration of joints aids in dx - Normal fluid is clear, viscous with WBC of 2000 cells/mm - RA -- fluid has poor viscosity and high WBC count Management of RA - Goal = remission or low disease activity achieved as rapidly as possible - Multifaceted treatment plan - Tx directed toward control of synovitis and prevention of joint injury - Non Pharm - PT/OT/psychological interventions - Regular exercise - Promote msk strength, functional ability and psychological well being - Pharmacologic - DMARDS = mainstay of tx and should start as soon as possible - Methotrexate = first line - Can cause folic acid depletion, supplement folic acid - Glucocorticoids -- may be useful at low dose - Avoid long term for side effect and ade profile - NSAIDS - May improve pain - Use w/ caution in pt with CV disease, renal disease, peptic ulcer Complications of RA - Joint deformity - Resulting sequela of muscle, tendon, ligament weakening or deconditioning - Joint immobility - Small vessel vasculitis = leads to neuropathy and skin ulcers - Complications related to adverse reactions to medications used in treatment - Osteoporosis, osteonecrosis, renal toxicity, GI irritation and bleeding, hepatic toxicity - Immunosuppressive state - RA patients associated w/ increased risk for lymphoproliferative disorders **Systemic Lupus Erythematosus (SLE)** - Autoimmune disease impacting skin, musculoskeletal, renal, cardiovascular, pulmonary, and reproductive systems - Unknown cause - Produces autoantibodies resulting in end-organ damage Patho - Hallmark of SLE - Development of antibodies directed against components of self tissues - Particularly structures found within cell nuclei - Autoantibodies form immune complexes in circulation and deposit in kidneys, skin, lungs and other target organs - Normally these are cleared by reticuloendothelial system but in SLE there is aberrant clearance generating local inflammation resulting in end organ specific effects Clinical Presentation of SLE - Varied presentation, disease relapses and remission - Arthritis, nephritis, serositis, vasculitis - May also have mild symptoms - Fatigue, arthralgia, rashes sporadically for years - May have anorexia and weight loss - May have headaches - Fevers, lymphadenopathy, tachycardia and anemia - Skin involvement - Malar butterfly rash - Photosensitive, erythematous rash on cheeks over bridge of nose that tends to spare nasolabial folds - Discoid lupus rash - Lesions are thick, round, erythematous plaques on face, scalp and extremities - Musculoskeletal involvement - Inflammatory arthritis - Milder than arthritis with RA, non erosive - Osteoporosis is common - Cardiac involvement - Chest pain is common complaint, often MSK in origin - Pleurisy, pericarditis, pneumonitis, interstitial lung disease, pulmonary hemorrhage, myocarditis, valvular heart disease - Vascular involvement - 1/3 of SLE will develop Raynaud phenomenon - Increased risk of vasculitis and thromboembolic disease - Renal involvement can develop lupus nephritis - Can be asymptomatic hematuria to glomerulonephritis with loss of renal fxn - Pulmonary involvement - Pleuritis, pneumonitis, interstitial lung disease, pulmonary HTN, alveolar hemorrhage - Neuropsychiatric involvement - Cognitive dysfunction, psychosis - Mood changes, depression and migraines - Ophthalmologic involvement - Retinal vasculopathy, uveitis - GI involvement - Majority of symptoms caused by mediation reaction and viral/bacterial infection - Hematologic abnormalities - Anemia of chronic disease - Leukopenia - Thrombocytopenia Diagnostics for Lupus - Dx of exclusion (have to rule out everything else to tell if someone has lupus) - During exacerbation -- lab tests reveal nonspecific evidence of systemic inflammation - Elevated ESR and CRP - CBC - Anemia is common - Leukopenia, lymphopenia, thrombocytopenia are characteristic - Urinalysis and renal function should be monitored closely - ANA test - \+ ANA is not specific but if negative ANA reduces likeliness of SLE - \+ ANA hallmark of SLE, should refer to rheumatology at this point of other testing Management of Lupus - Avoid prolonged sun exposure (many pt are photosensitive) - Modest activity - Diet can help manage risk factors (atherosclerosis and metabolic syndrome) - May require increased caloric intake - Glucocorticoids for management can increase appetite and cause weight gain - Vitamins are needed (Vit D with sun avoidance) - No specific food or diet has shown to trigger SLE - Low dose omega 3 fatty acid has shown to have benefit on disease activity and vascular function - Cardiovascular risk reduction = statin therapy when indicated - Monitor calcium and vit D levels (frequent occurrence of osteoporosis) - Increased incidence of cervical dysplasia and HPV (regular gynecologic care) - Consideration for abx prophylaxis prior to dental procedures - Increased risk for endocarditis especially in those with valvular heart abnormality Pharmacologic for LUPUS - NSAIDS -- pain treatment - Hydroxychloroquine - Manage musculoskeletal, cutaneous and serosal manifestations of disease - Corticosteroids = MAINSTAY OF TREATMENT - Immunosuppressive agents may help control disease and taper corticosteroids - Dx and management by rheumatology is recommended **Vasculitis** - Group of disorders where there is inflammation and damage to blood vessel walls leading to tissue necrosis - Often have multi organ dysfunction - Conditions are serious, often fatal if not recognized early and treated aggressively - For most conditions -- age of onset is variable ranging from infancy to old age - Some vasculitis only impacts certain age groups - Classification of vasculitis syndromes - According to size of blood vessels involved - Large vessel vasculitis - Giant cell arteritis = most common of all vasculitis - New headache - Elevated ESR - Abnormal artery biopsy - Temporal artery abnormality/tenderness - Takayasu arteritis - Medium vessel - Polyarteritis Nodosa - Necrotizing arteritis thar impacts medium but also small arteries - Destroys vessel walls causing development of aneurysms - Common features - Ulcerating Skin lesions - Hypertension (renal involvement) - Postprandial abd pain from mesenteric artery involvement - Testicular or ovarian pain from gonadal artery involvement - Peripheral neuropathy - Kidneys, skin, joints, muscles, nerves and GI tract are commonly involved - Wegener Granulomatosis aka granulomatosis w/ polyangiitis (GPA) - Classic Triad of involvement - Upper airway - Nasal obstruction and rhinorrhea - Epistaxis - Watery eyes - Sinusitis - Recurrent OM - Lower airway - Single or multiple cavitary nodules on film - Persistent cough/hemoptysis or dyspnea - Kidneys - Nephrotic syndrome - Hematuria, proteinuria, hypertension, uremia - Small Vessel - Patho of vasculitis - Classification - Primary - Idiopathic - Autoimmune etiology - Secondary - Infection - Hep B and C, TB, syphilis - Connective tissue diseases - SLE, RA, mixed connective tissue diseases - Drugs - Hydralazine, PTU, sulfonamides, betalactams, quinolones - Clinical presentation of vasculitis - Most vascular syndromes have characteristic clinical, laboratory, or pathologic features that allow for identification and classification - Constitutional symptoms - Fever - Malaise - Anorexia - Weight loss - Arthralgias - Myalgias - Diagnostics and Management of Vasculitis - Basic lab studies including inflammatory studies - CMP, CBC, CRP/ESR - Patients w/ suspected vasculitis should be referred to rheumatology for definitive diagnosis and workup - Treatment is dependent on type and severity of the vasculitis **Sjogren's Disease** - Systemic autoimmune disease with gradual progressive lacrimal, salivary, and exocrine gland dysfunction - Presents as dryness in all areas of the body where there are exocrine glands associated with mucus membranes (most notably the salivary and lacrimal glands) - Cause = unknown (estrogen may play a role as disease more common in women) - More common in patients with other autoimmune disease - 25% of patients with RA and SLE are impacted Clinical Features of Sjogren's Disease - Ocular - Keratoconjunctivitis (eye dryness) - Causes a prolonged but slowly progressive decrease in tear production - Increased risk for corneal abrasion or eye infection - Characteristic symptoms - Insidious onset of ocular foreign body sensation, burning pain, inability to tear or photophobia - Some pt may be asymptomatic - Physical Exam - May have conjunctival injection - May have slit lamp exam abnormalities - Cornea may have dull appearance - Treatment = artificial tears - Oral-Salivary - Decreased saliva production - Qualitative changes in the saliva and oral flora - Increased risk for dental caries - Characteristic symptoms - Dry mouth (xerostomia), difficulty chewing or swallowing food, difficulty wearing a lower denture, oral burning - Some may be asymptomatic - Physical exam - Decreased salivary pool - Dry mucous membranes - May progress to erythema, fissuring, and ulceration - May have multiple dental caries - Treatment = chewing gum (sugar free) to reduce dental caries risk Diagnosis and Prognosis of Sjogren's Disease - Multitude of objective tests help with diagnosis - Ophthalmologic eye evaluation - Salivary gland and saliva production testing - Autoantibody testing - Initial lab testing = CBC, RF, ANA - Most serious complication - Development of lymphoproliferative disease - Primary Non-Hodgkin lymphoma **Fibromyalgia** - Most common cause of chronic, widespread pain in the US - Etiology is unknown and patho is unclear - Considered to be a disorder of pain regulation - ACR classification criteria for fibromyalgia (not diagnostic) - Must have hx of chronic widespread pain - Involves all 4 quadrants of the body - Axial skeleton - Presence of 11 of 18 tender points on physical exam A screenshot of a diagram of a person\'s body Description automatically generated Clinical Features of Fibromyalgia - Characterized by - Chronic, widespread MSK pain with fatigue - Numbness, tingling and other abnormal sensations - Often accompanied by cognitive and psychiatric disturbances - Fatigue and poor sleep - "fibro fog" = issues with attention and difficulty doing tasks that require rapid thought changes - Anxiety and depression - Some disorders are seen in greater frequency in patients with fibromyalgia - IBS and migraines - Cardinal manifestation is widespread pain - Pneumonic for Fibromyalgia features = FIBROw - F -- Fatigue (chronic) - I -- Insomnia, irritability, irritable bowel/bladder - B -- Blues (anxiety and depression) - R -- Rigidity (muscle and morning joint stiffness) - Ow -- Pain, widespread and chronic - Tender points, paresthesia, temperature changes, migraine, feeling of swollen joints, panic attacks, memory lapses, concentration deficit Diagnosis of Fibromyalgia - History and physical exam - Limited lab testing usually done to exclude other conditions - CBC - ESR and or CRP (should be negative as this is not an inflammatory condition) - ANA and RF should be obtained if hx and physical suggest inflammatory, systemic rheumatic disease - Thyroid testing or CK if inflammatory muscle disease suspected Management of fibromyalgia - Education - Nature of disorder - Discussion should include expectation for tx and symptom prioritization - Consider symptom diary - Pharmacologic - Antidepressants - TCAs - Reduce pain, fatigue and sleep disturbances - Considered first line tx - SSRI/SNRI - Pregabalin and gabapentin may be useful adjunct of treatment for pain - Avoid opioids and steroids (not inflammatory) - Aerobic exercise - Pain and functionality have shown to improve with regular exercise of moderate intensity - Complimentary and alternative therapies - Massage, hydrotherapy, acupuncture, homeopathy - None showed consistent benefit but may help some patients **Sarcoidosis** - Multisystem granulomatous disease of unknown etiology - Impacts lungs, intrathoracic lymph nodes - Can involve any organ of the body - 80% of cases occur in adults between 20-50 years of age - Second peak incidence between 50-65 years of age - Lifetime risk of sarcoidosis in US is greater in African Americans Pathogenesis and Etiology - Non-caseating granuloma formation due to ongoing inflammation - Precise cause is unknown - Most studies suggest it is a result of exaggerated immune response in a genetically susceptible individual, to an undefined antigen Clinical presentation - Pulmonary = dyspnea, cough, wheezing, chest pain - Upper airway = dyspnea, nasal congestion, hoarseness, stridor, polyps - Dermatologic = nodules, papules, plaques - Ocular = photophobia, tearing, pain, decreased visual acuity, lacrimal gland enlargement, uveitis, glaucoma, blindness - Rheumatic = polyarthropathy, monoarthropathy, myopathy - Neurologic = headache, hearing loss, paresthesias, seizures, cranial nerve palsy - Cardiologic = syncope, dyspnea, arrythmias, CHF, Tamponade - GI = dysphagia, abdominal pain, jaundice, hepatomegaly - Hematologic = lymph node enlargement, hypersplenism - Renal = Kidney failure, calculi Diagnosis and treatment - Dx - Compatible clinical and radiologic presentation - Stage 0 = normal chest radiograph - Stage 1: bilateral hilar lymphadenopathy - Stage 2: BHL with pulmonary infiltrates - Stage 3: pulmonary infiltrates without BHL - Stage 4: Pulmonary fibrosis - RF is often positive - Anemia may be present - Leukopenia, eosinophilia, thrombocytopenia can be seen - ESR is often elevated -- nonspecific finding - Often need tissue biopsy - Treatment - Not indicated for pt with asymptomatic stage 1 and 2 sarcoidosis - Tx with corticosteroids is mainstay for more severe stages of disease - May resolve spontaneously in 1-2 years time **Juvenile Idiopathic Arthritis (JIA)** - AKA stills disease or systemic juvenile rheumatoid arthritis - Most common form of childhood arthritis - Cause = unknown - Dx requires persistent arthritis for more than weeks in pediatric patient younger than 16 years old Clinical Features - Pain (mild to moderate aching) - Joint stiffness (worse in AM and after rest) - Joint effusion and warmth - Systemic symptoms are more common in systemic and polyarticular subtypes - Anemia, anorexia, fever, fatigue, lymphadenopathy, weight loss - Growth abnormalities can result in growth disturbances - Premature fusion of epiphyses, bony overgrowth and limb length discrepancies Physical Exam Findings - Swelling of joint with effusion or thickening of synovial membrane - Heat over inflamed joint and tenderness along joint line - Loss of ROM and function - Nonmigratory or monoarticular or polyarticular involvement of large or proximal interphalangeal joints for 3 months - May have systemic manifestations = rash, fever, leukocytosis, lymphadenopathy - Dx - Of exclusion - Based on physical exam findings and hx of arthritis lasting longer than 6 weeks - No diagnostic test - Most have negative inflammatory markers Treatment of JIA - NSAIDS - Mainly those with oligoarthritis - Do not alter disease course or prevent joint damage - Used to tx pain and stiffness and fever associated - Corticosteroids - Minimal use due to impact on bone and growth/health - Methotrexate - Cornerstone of medical management - DMARDS and Anti-TNF meds are beign used for cases refractor to MTX therapy - \*\*\* MANAGEMENT done with pediatric rheumatology Meds used in rheumatology - Adverse effects of corticosteroids - Common in pt receiving drugs in high doses for prolonged periods - Major ADE arise from inhibition of HPA function and development of iatrogenic Cushing's syndrome - Skin and soft tissue - Skin thinning and purpura (often sun exposed areas) - Non melanoma skin cancers more common - Steroid acne - Hypertrichosis - Striae - Cushing appearance and weight gain - Cushingoid features - Truncal obesity, buffalo hump, moon face - May appear in first 2 months of therapy - Increased appetite may cause increased food intake - Eye - Increased risk of cataracts and glaucoma - CV disease - Associated with increased risk of serious adverse CV affects - Ischemic heart disease and heart failure - GI - Gastritis, ulcer formation, GI bleeding and pancreatitis - Avoid concurrent use with NSAIDS when possible - Prevent GI toxicity -- prophylaxis - Add PPI - Using selective Cox 2 inhibitor with or without PPI Risk factors for GI toxicity - High risk - Hx of complicated peptic ulcer disease - Multiple \>2 risk factors - Moderate risk (1-2 risk factors) - Age \>65 - High dose NSAID - Hx of uncomplicated ulcer - Concurrent use of ASA (including low dose), glucocorticoids, or anticoagulants - Low risk - No known risk factors - Kidney and system hemodynamics - Promote fluid retention (may be issue for those with underlying CV or renal disease) - Raise BP - GU - May cause menstrual irregularities in females - Musculoskeletal - Osteoporosis - Vertebral fractures (increased bone fragility) - Osteonecrosis - Growth impairment in kids - Msk weakness - CNS - Mood disorders - Psychosis - Memory impairment - Akathisia - Pseudotumor cerebri - Glucose metabolism - Dose dependent mild increase in fasting glucose - Infection and immune response DMARDS - May be biologic or non-biologic - Biologic agents - Monoclonal antibodies and recombinant receptors - Block cytokines that promote the inflammatory cascade responsible for RA symptoms - TNF inhibitors - First line therapy - Adalimumab (humira) - Etanercept (Enbrel) - Infliximab (remicade) - Non-biologic - Methotrexate - First line treatment - SE: Liver, teratogenesis, hair loss, oral ulcers - Leflunomide (Arava) - SE: liver, GI, teratogenesis - Sulfasalazine (Azulfidine) - GI affects, anemia in G6PD deficiency - Hydroxychloroquine (Plaquenil) - Rare ocular toxicity