PMSB Quiz 5 PDF

Venous Disease - DVT and PE What is DVT - Very common - Thrombus in deep vein involving venous valve cusp, venous branch points of calf, thigh or pelvis - A blood clot forms in the deep veins of the extremities, most commonly in the leg...

Venous Disease - DVT and PE What is DVT - Very common - Thrombus in deep vein involving venous valve cusp, venous branch points of calf, thigh or pelvis - A blood clot forms in the deep veins of the extremities, most commonly in the leg but can occur in the arms and mesenteric and cerebral veins - It is considered part of the venous thromboembolism disorders - It represents the 3rd most common cause of CV deaths after MI and stroke - Hospitalized pt are prime ppl to have DVT Etiology (causes) of DVT Associated with Virchow’s triad of: Hypercoagulopathy, Stasis, and Endothelial Damage HYPERCOAGULOPATHY (exaggerated coagulation) - Increased circulating tissue activation factor - Decreased plasma antithrombin and fibrinolysis - Ask family hx, “have you ever had a blood clot before?” STASIS - From any mechanism retarding or obstructing venous return - “I just came back from europe and now my leg is very tender” ENDOTHELIAL DAMAGE - Surgery, external injury, vein catheters, smoking, HTN DVT H&P The clinical presentation of DVT varies with the: - Anatomic distribution - Extent - sometimes it can look very minor - Degree of occlusion of the thrombus - Can range from neuropathy to even systemic pain Symptoms may range from - Absence to massive swelling - Cyanosis with impending venous gangrene There are 3 patterns of Thrombosis: - Isolated calf vein (distal) - Femoropopliteal - Iliofemoral thrombosis ONLY 50% of patients present with pain In their P.E → limb edema may be uni or bilateral of the thrombus extends to pelvic vein ***a lot of times, with severe venous insufficiency it will present bilaterally Hx of redness In their P.E → red and hot skin with dilated veins ***not always the case 70% of patients present with swelling In their P.E → tenderness DVT DDx - Cellulitis - Bakers cyst - Soft tissue injury to calf - abdominal/pelvic tumors - Venous HTN - Hypoalbuminemia ***ALWAYS GET A VENOUS DUPLEX ULTRASOUND - ITS NONINVASIVE, DOES NOT HARM PT, BENEFIT OUTWEIGHS RISK DVT risk factors - Male - > 45 - Prolonged bed rest/hospitalization - Long flights - Central venous catheters - Tobacco use - Sickle cell anemia - Polycythemia - CHF DVT signs & symptoms Most common - Leg pain, edema, erythema, tenderness, low grade fever, prominent superficial veins - Pain with passive dorsiflexion of the foot (Homan’s sign)* - Peripheral cyanosis ***50% of pt with acute DVT may lack specific signs & symptoms Post-op patients are more likely to have small, asymptomatic, distal, non-occlusive thrombi DVT inherited vs acquired Inherited DVT - Factor V leiden - Prothrombin gene mutation G20210A - Antithrombin deficiency - Protein C deficiency - Protein S deficiency Acquired DVT - some diseases make you hypercoagulable - Cancer - Oral contraceptives (obese pt who just had baby) - Hormone replacement therapy  - Pregnancy - Obesity - Heparin-induced thrombocytopenia DVT Complications Pulmonary embolism, death in 10-30% of pt within 1 month of diagnosis, venous ulceration (medial or lateral malleoli) ulcers make it a chronic problem Diagnostic/Lab Testing for DVT D-dimer - used as initial screening in ER, provides fast and cost effective way to triage pt, 97% specific, increased levels 7 days post DVT Venous ultrasound (duplex ultrasonography) - 90% sensitive, 95% specific for femoral, popliteal vein thrombosis; 50% sensitive for calf DVT Contrast venography MRI - alternative to CT CT - may detect smaller emboli Impedance plethysmography CONTRAST VENOGRAPHY IS THE GOLD STANDARD - Difficult to perform in pt with edema or obesity due to impaired venous access Treatment plan for DVT with Pharmacological Treatment Indefinite treatment for recurrent DVTs, continued risk factor Anticoagulation to prevent further thrombosis, PE, post-phlebitis syndrome, chronic thromboembolic pulmonary HTN **transient risk usually for 3 months **idiopathic risk for 3-6 months Pharm treatment: - Fondaparinux Anticoagulant med to prevent thromboembolism to treat DVT and to improve survival after MI - Unfractionated Heparin IV infusion - Low molecule weight heparin Outpatient treatment, commonly used as bride with warfarin, reduced dose in renal pt - Dabigatran Treats DVT and PE in adults and kids 3 mo+ who’ve been treated w injectable blood thinner - Warfarin Dual therapy due to delayed depletion vitamin K dependent coagulation factors - Rivaroxaban, apixaban Monotherapy, no coagulation therapy Non-pharmacological Interventions for DVT IVC, graduated compression stockings, intermittent pneumatic compression (IPC)  DVT Prophylaxis: Orthopedic Surgeries DVT and PE high risk in patients undergoing major orthopedic surgeries like knee or hip surgeries Total hip arthroplasty - Low molecular weight heparin - Apixaban - Rivaroxaban Hip fracture surgery use - Low molecular weight heparin - Unfractioned heparin - Fondaparinux DVT Prophylaxis: Non-orthopedic Surgeries Major surgeries are categorized as moderate to high risk for VTE and need DVT prophylaxis - Low molecular-weight heparin is preferred, but unfractioned heparin is used in patients with renal insufficiency - Fondaparinux used in patients with heparin induced thrombocytopenia - Duration: few days or until patients can ambulate of discharge from the hospital Pulmonary Embolism A life-threatening manifestation of venous thromboembolism that can be challenging to diagnose and manage **REMEMBER DVT IS A CAUSE OF PE It occurs when a portion of the clot from a DVT breaks off, travels to the right heart and eventually lodges in the pulmonary vasculature PE H&P A proper H&P exam is necessary → establishes accurate diagnosis of PE bc the symptoms are very variable Symptoms depend on the severity of the disease ranging from mild dyspnea, chest pain, and cough to sustained hypotension and shock Many patients are asymptomatic Sudden death can be the initial presentation!!!! PE ddx - Acute coronary syndrome - Stable angina - Acute pericarditis - CHF - Malignancy - Cardiac arrhythmias - Pneumonia, pneumonitis, pneumothorax - Vasovagal syncope  PE risk factors - Surgery - Cancers - Heart and lung disease - Broken leg or hip bone/other trauma - Hormone-based meds - Pregnancy and childbirth - Immobility (long flight) - Age, fam hx and genetics, obesity PE signs & symptoms - Tachypnea - Tachycardia - Similar signs to DVT: calf/thigh swelling, erythema, edema, tenderness, palpable cords - Rales / cough - Decreased breath sounds / SOB at rest or effort - Accentuated pulmonic component of the second heart sound - Jugular venous distension → veins in neck may be pulsing - Orthopnea (SOB when lying supine) - Pleuritic chest pain Clinical scores to estimate probability of PE PE Inherited vs Acquired Inherited - Factor V Leiden - Prothrombin gene mutation - Antithrombin deficiency - Protein C deficiency - Protein S deficiency - Hyperhomocysteinemia Acquired (Non-provoking) - Age - Venous insufficiency - Obesity - Rheumatologic disease - Previous venous thromboembolism - Antiphospholipid antibody syndrome (Provoking) - Surgery and trauma - Cancer - Prolonged immobility  - Estrogen use & pregnancy - Indwelling catheter PE Biomarkers D-dimer - Extremely sensitive biomarker of acute PE - Formed as result of acute thrombosis and is a fibrin degradation product D-dimer usage - Low and intermediate probability for PE - High probability of PE Troponin - Troponin I and T levels: most sensitive and specific biomarkers of myocardial injury Natriuretic peptides - Valuable in evaluating a hemodynamically significant pressure on the right ventricle due to large PE Pharmacological Support for PE Vasopressors - Increase SVR and eventually MAP and prevent RV ischemia Inotropes - Secondary to the use of vasopressors and is mainly aimed at resolving a low CO state after BP has been stabilized Pulmonary arterial vasodilation - Inhaled nitric oxide - inducing an increase in CO in pt w increased right ventricular afterload Management of High-Risk PE Respiratory support - Aggressive management of hypoxemia and hypercapnia - Oxygen supplementation - High Flow Nasal Cannula (HFNC): non-invasive method of administering high oxygen concentration and flows Fluid Management - Administration of fluids for the resuscitation of patients in shock is common and prevalent Hemodynamic Support - Judicious fluid resuscitation - Inotrope / vasopressor support - Correction of underlying cause Surgical Pulmonary Embolectomy (SPE) Option for patients with submassive PE at high risk for adverse outcomes or with contraindications to fibrinolysis First line treatment rather than as rescue therapy Indications:  - Contraindication to lytic therapy - Clot in transmit - Large PFO or ASD - Moderate to severe right ventricular dysfunction - Failed catheter directed or systemic thrombolysis Chronic Venous Insufficiency - Classified as either primary or secondary to DVT - Aka: stasis dermatitis, postthrombotic syndrome, chronic venous disease - Refers to: lower extremity edema, skin trophic changes, discomfort secondary to venous hypertension Pathophysiology High venous pressure in the lower due to failure in keeping venous upward flow toward the heart Can result in backward reflux of venous flow through incompetent valves in the deep or superficial venous systems Venous pressure is elevated, and blood return is impeded (delayed) - Valvular incompetence - Thrombotic or non-thrombotic venous blockage - Extrinsic compression of the veins Symptoms - Achy or tired legs - “Pins & needles” - Cramping in legs at night - Discolored skin that looks reddish-brown - Edema (swelling) in lower legs and ankles - Flaking or itching skin on legs or feet - Full or heavy feeling to the legs - Leathery-looking skin - Ulcers near the ankles - Varicose veins Attribute Absent = 0 Mild = 1 Moderate = 2 Severe = 3 Pain None Occasional pain Daily pain or Daily pain or or other discomfort discomfort that limits discomfort most daily activities Varicose veins ≥3 mm in None Scattered, Confined to calf Involves calf and diameter includes corona or thigh thigh phlebectatica Edema None Limited to foot & Extends above Extends to knee and ankle ankle but below above knee Pigmentation None or focal Limited to Diffuse over Above lower third of perimalleolar lower third of calf calf area Inflammation None Limited to Diffuse over Above lower third of perimalleolar lower third of calf calf area Induration None Limited to Diffuse over Wider distribution perimalleolar lower third of calf above lower third of area calf # of active ulcers 0 1 2 ≥3 Durations of longest active None 3 mo but 1 yr ulcer Size of largest active ulcer None Diameter 6 cm cm Compression therapy Not used Intermittent use Wears stockings Full compliance: of stockings most days stockings Clinical-etiology-anatomy-pathophysiology (CEAP) classification system How bad is this ulceration? Class Clinical signs 0 No visible or palpable signs of venous disease 1 Telangiectasis or reticular veins 2 Varicose veins 3 Edema 4 Skin changes ascribed to venous disease 4a Pigmentation, venous eczema 4b Lipodermatosclerosis, atrophie blanche 5 Skin changes as defined above, with healed ulceration 6 Skin changes as defined above, with active ulceration Diagnostic Testing: CVI - Duplex venous ultrasound of lower limb - Plethysmography - CT venography  - MR venography - Contrast venography - Intravascular ultrasound - Ambulatory venous pressure Ddx for CVI (DERMATOLOGICAL ISSUE) - Contact dermatitis - Atopic dermatitis - Cellulitis - Dermatophyte infection - Pretibial myxedema - Nummular eczema - Xerosis - Asteatotic eczema Differences: venous, ischemic, and neuropathic ulceration Patient History Venous stasis ulcer Ischemic (arterial) ulcer Neuroischemic ulcer Rapid onset Slow, progressive Diabetes Edema Arteriosclerosis Peripheral neuropathy Trauma Claudication Thrombophlebitis Age usually >45 Significant smoking hx  HTN Hyperlipidemia Pain Venous stasis ulcer Ischemic (arterial) ulcer Neuroischemic ulcer Some pain Moderate to severe Neuropathy (not painful) Increases with dependency Intermittent claudication Anesthesia Decreases with elevation Decreases with Paresthesia dependency Ischemic with elevation (at night) or leg exercises Location Venous stasis ulcer Ischemic (arterial) ulcer Neuroischemic ulcer Medial malleolus Lateral malleolus Pressure sites Ankle Anterior tibia Plantar surface Lower calf Toes, heels, bony Heels, bony prominences prominences Stocking distribution Appearance Venous stasis ulcer Ischemic (arterial) ulcer Neuroischemic ulcer Irregular border Well demarcated, punched Thin, undetermined border out Base with granulation tissue Pale or white base Black, gray, or yellow base Exudative Weeping Surrounding Skin Venous stasis ulcer Ischemic (arterial) ulcer Neuroischemic ulcer Brown pigmentation Dry eschar Pale Hyperkeratotic borders Pale, cyanotic Reticular vascular pattern Edema Cool Palpable purpura Mottling Thin Hemorrhagic vessels Stasis dermatitis Shiny Callus around wound Dependent rubor Bullae formation Hairless Charcot’s deformity Hammertoes Vascular exam Venous stasis ulcer Ischemic (arterial) ulcer Neuroischemic ulcer Pulses may be normal Pulses decreased to absent Pulses usually present Normal ABI (>0.9) Low ABI ( 3.5 g.dl - Total lymphocytes > 1500mL - Abnormal low transferrin, prealbumin, retinol binding protein levels - Elevated HbA1C - Documented hx of same side infection requiring long-term antibiotics - Active same site infection/remote infection - Active same site poor wound healing - DIFFICULT REVISION SURGERY **Multiple RA meds + smoking + alcohol abuse + elevated HbA1C Principles to Procedure Selection in the Rheumatoid Foot RA is an inflammatory, chronic condition that causes profound changes in the function and structure of the foot & ankle Foot and ankle problems becomes increasingly common in the rheumatoid patients with time Progression of the disease leads to a pronounced inflammatory, deformity and compromised function of the FF, MF, RF especially if conservative medical modalities are unable to control the disease The goals of surgery for RA patients are different than those a healthy, normal patient MSK Presentation Symmetrical, inflammatory arthritis, joint pain, Cervical spine involvement redness & warmth Pannus → synovial expansion that destroys articular cartilage Morning stiffness for 1-2 hrs, gets better with motion, Felty’s syndrome progressively more painful throughout the day Swan neck deformity → hyperextension of the PIPJ Pain gets worse after rest and flexion of the DIPJ Boutonniere deformity → flexion of the PIPJ and hyperextension of the DIPJ (same to hammertoe) Mostly seen in 4th and 5th MTPJ of the foot Ulnar (lateral) deviation of toes, pes planus, hallux Subluxation of the MTPJ abducto-valgus deformity Anterior shift of FF pad Rheumatoid nodule → painless nodule over bony pro Baker’s cyst → synovial fluid cyst in popliteal fossa Increased TBI → TUNICA MEDIA CALCIFICATION Ulcerations due to deformity  H&P Examination Initial assessment: detailed hx taking and thorough clinical presentation Important to identify patient expectations and common presenting complaints include deformity with or without pain, difficulty in shoe wear, decreased mobility and balance Focus on: - Deformity of first ray (hallux valgus) and its ability to perform WB mechanical function - Prominence of a painful bunion with or without inflamed bursa, flexible or rigid - Deformities of lesser toes (asses if flexible or rigid, claw/hammertoes with painful callosity formation over the dorsum of the interphalangeal joints and under exposed MT heads - Assess quality of soft tissue - Gait assessment and power of hallux tendons Indications for surgery Failure of non-surgical measures Painful bunion or painful still first MPJ, combined with pain under the lesser MT heads from synovitis Destruction of the plantar plate or frank dislocation of the MTPJ Hammertoes or mallet toes or ulceration/calluses Any foot or ankle worsening deformity OR deformity interfering with shoe wear, mobility, gait Pre-op surgical conditions DMARDs Cardiac conditions Vascular status Cervical spine disease Fragile non-elastic skin Fragile vessels easily bleed (anticoagulants) Thin capsule and weak tendons Bone condition - erosions, osteoporosis Difficulty with crutches or walker Types of Surgical Management Pan-metatarsal head Resections & Soft tissue Fusion & implants resection (HLC) osteotomies Hoffman Keller Tenotomy 1st MTPJ fusion Larmon Scarf Synovectomy 1st MTPJ silastic implant Clayton Hohman Digital arthrodesis Chevron Lapidus Weil Midfoot arthrodesis Digital arthroplasty Ankle fusion STJ fusion Triple arthrodesis Pan-talar fusion Specifics on Surgical Management First metatarsal Hallux resections Fusion Implants osteotomies Scarf Keller 1st MPJ Silastic implants Hohman fusion/arthrodesis Chevron Ex: Swanson hinges, Hemi-arthroplasties, Spacer devices Indicated in mild to Proximal phalanx GOLD STANDARD: Preserve the joint mod hallux valgus base resection Indicated for severe and provide pain hallux rigidus and relief Becoming more Shorter, less active pt popular in treatment complicated Indicate hallux of RA FF Provides long-term valgus with mild to Now is reserved as maintenance of moderate arthritis, Provides stable a joint salvage structure & functional younger, active correction of procedure correction individuals deformity along w maintaining mobility Used in older, less Increased stability not *fell out of favor due of the joint active pt with pain & only at MPJ but to hella HAV deformity across entire FF complications in ppl w RA Complications: Complications: Complications: Complications: Recurrence Stress fx of 2nd toe Delayed or nonunion Osteolysis & Cock-up deformity Removal of painful loosening Flail toe hardware Granulomatous rxn to Transfer silicone metatarsalgia Breakage Proximal migration of sesamoids Pan Metatarsal Head Resection #1 PROCEDURE FOR TREATMENT OF RA Hoffman Larmon Clayton Hoffman Transverse plantar skin incision Wedge removal of fat Minimal neurovascular damage Resection of all 5 mt heads Pull digits plantar Larmon 3 dorsal incisions Only partial mt head resections and plantar condylectomies  Clayton *same like Hoffman Except - Generous resection of all 5 mt heads AND resection of 4 bases of the proximal phalanges CONSIDERATIONS for pan met head resection - Amount of bone resection = amount necessary to relieve all soft tissue tension - If not enough bone is removed = too much tension = vascular compromise = lose toes? - Multiple toes involved = panmetatarsal head resection - Stiff, stable, rectus toes - Even distribution of FF wb COMPLICATIONS for pan met head resection - Digital vascular compromise (dry gangrene) - Toe loss and amputations common - Recurrent prominent plantar lesions - Non-purchasing elevated toes - Second or third surrey expected and common Lesser digits surgery Indicated for hammertoes and claw toe Complications deformity Non-union, loosening, migration, revision Often used in combo with metatarsal surgery A flexible deformity can be treated with A rigid or fixed deformity can be treated with - Selective tenotomy or arthroplasty - Arthrodesis with K-wire fixation - Material metal devices such as smart toe - Closed osteoclasis → break the toe at deformity Midfoot and Hindfoot deformity Lapidus, ankle & stj arthrodesis, tripple, pan-talar fusion Patients with RA develop destructive midfoot and ankle diseases that lead to: - Instability - Collapse - Degenerative changes Indicated - Midfoot deformity due to degenerative changes or collapse - End stage for pes planovalgus collapse - Pain with moderate to severe midfoot and ankle arthritis  Rheumatoid Foot 1 & 2 Know the etiology and epidemiology of RA Chronic inflammatory joint disease seen in clinical practice affecting approx 1-3% of population Characterized by persistent inflammatory synovitis leading to cartilage damage, bone erosions, joint deformity and disability Patients most commonly affected in 3rd to 6th decide - F to M is 3:1 Morning joint stiffness >1 hour and easing with physical activity is characteristic Know the clinical manifestations of RA Pannus - formation of inflammatory granulation tissue that lead to the destruction of cartilage and bone ultimately causing deformity of the joint Pre-clinical stage: muscle pain, tiredness, weight loss, generalized lack of well being “just dont feel well” Early stage: - Polysynovitis: pain, swelling, stiffness of MCP, PIP joints then to wrist, feet, knee, shoulder - Tenosynovitis: wrist extensors, finger flexors common - Morning stiffness > 1 hr - If large joint involves: local warmth, synovial hypertrophy, effusion, restricted movements Later stage - Deformity become apparent (xrays come in handy) - Acute pain of synovitis replaced by constant ache pain of joint destruction - Joint instability and tendon rupture - Rheumatoid deformities - Restricted movements of joints - ⅓ report pain and stiffness in cervical spine Know the extra-articular manifestations of RA Constitutional symptoms - FEELING LOUSY Rheumatoid nodules in up to 30% of pt - common in elbows and dorsum of hands - Common and seen in more severe cases of RA - Its close to pressure points, intact overlying skin, firm to hard, and can be fixated to periosteum - Biopsy rarely needed Hematological - Normocytic normochromic anemia - Leucocytosis/Leucopenia - Thrombocytosis Felty’s syndrome - triad - Chronic nodular RA (sero+) - Rheumatoid nodules - Spleenomegaly - Neutropenia - have too many WBCs Know imaging features of RA including the survey and common joints affected Rheumatoid nodules are - Benign and v common so a biopsy is rarely needed - Seen in severe cases Rheumatoid nodules in ultrasound: - HYPOECHOIC mass (lack of echo, well-circumscribed, no vascular or blood vessels going to nodule) - No doppler - NO POSTERIOR ACOUSTIC SHADOW *no shadow deep to the nodule bc the nodule is soft and jelly-like so it does not block the ultrasound and create a shadow Joint involvement In hands it is seen more proximal, in feet it is seen more distal so usually the MPJ except for sometimes the hallux you see it in the proximal phalanx CERVICAL SPINE INVOLVEMENT IS SEEN - take a cervical spine xray Imaging NOT FEASIBLE TO GET RADIOGRAPHS OF ALL JOINTS - AP and oblique of both hands and wrists - AP, mortise, and lateral of both ankles - DP, MO, lateral of both feet - PA of both knees - Cervical flexion of the spine - Sometimes take a pelvis and chest view as well **note the ulnar/fibular drift - Carpals and metacarpals drift medial and phalanges drift lateral In xrays - We see erosions of the 5th mt lat head bc high peak pressure, high GRF Know labs associated w RA diagnosis including RF, anti-CCP, sed rate, CRP, and CBC RF - IgG (usually) antibody - Not all ppl with RA test +; some people who test + are Sjogren’s, and the seronegatives - 85% of pt will become RA positive over the first 2 years so repeat the test every 4-6 months - High values of RF mean poor prognosis and more extra-articular manifestation Anti CCP - Antibody to inflamed synovium - Has same sensitivity to RF but a higher specificity - It correlates when erosive damage occurs, joint damage occurring when its + - It is positive and high in severe cases Acute phase reactants - We don't usually order these, rheumatologist does  - They are proteins formed in response to inflammation; used as biomarkers Other tests Sed rate - shows inflammation; but it's an indirect measurement of reactants but is also somewhat precise - ACR REMISSION CRITERIA: ESR or equal to 6 weeks Must have > or equal to 4 to meet RA diagnosis Know the epidemiology, etiology, and common signs & symptoms of fibromyalgia, polymyalgia rheumatica, and Sjogren’s Fibromyalgia Central pain disorder; paresthesias, disturbed sleep, high fatigue Female disease; 9:1 Symptoms - Widespread pain for at least 3 months - Cervical area pain (consistent with both RA and fibromyalgia) - Headache, dysmenorrhea, extreme sensitivity to cold, intolerance to exercise, stiffness, paresthesia, easy fatigability, chronic sleep disturbances, impaired concentration, problems with memory, inability to multi-task, diminished attention span, anxiety & depressive symptoms ACR diagnostic criteria Changed from pinpoint to widespread **the tender point test is being replaced with a widespread pain index (WPI) and symptom severity (SS) score Impact on work & productivity QoL issue - ppl usually take a few days off work a month or receive disability/social security payments Pain, fatigue, depression are likely to contribute to sedentary lifestyles and therefore low levels of fitness → they don't feel like doing anything In moderation should work out Polymyalgia Rheumatica Characterized by severe pain and stiffness in neck Shoulder girdle and pelvic girdle!!!!! *PROXIMAL MUSCLE INVOLVEMENT HOWEVER, NO MUSCLE DISEASE IS PRESENT AT BIOPSY/MUSCLE STRENGTH IS NORMAL → emg is normal It is not a life-threatening disease but it does require treatment for 2-4 years - NO muscle atrophy - Muscle tenderness - Decreased active ROM of joints secondary to pain Sjogren’s syndrome Syndrome characterized by dry eye, dry mouth, muscle and joint pain, severe fatigue The cause of this condition is unclear, but involves immune system-mediated inflammatory mechanism - Dry eyes & dry mouth → glandular problem Etiology - Female:male is 9:1 - Autoimmune: HLA-B8/DR3 although DR4 is more closely associated with syndrome occurring with raynaud’s phenomenon - ANTIBODIES TO RO ANTIGEN OCCUR IN EXCESS IN RELATIVES OF PATIENTS WITH SJOGREN’S SYNDROME Epidemiology - Affects 0.1-0.3% of world population - NOT COMMON - No significant regional variations - Female predominant (90%) - Onset = 40 to 60 yrs of age but symptoms often present for many years Skin manifestations Xeroderma, pruritus, scaling Annular erythema, papular erythema including swees-like lesions Raynaud’s phenomenon Hyperglobulinemic purpura Vitiligo Sweating abnormalities Cutaneous amyloidosis ALOPECIA - diffuse and generalized Clinical features - extra glandular (systemic) Nearly all systemic can be affected but the commonest ones are: - Skin - joints/muscles - Lugs - Kidneys  - Neurological AUTONOMIC DYSFUNCTION DISABLING FATIGUE - 70% PSS pt with severe fatigue Know the lab testing for fibromyalgia, polymyalgia rheumatica and Sjogren’s Fibromyalgia Electromyographic biofeedback training may therefore be a therapeutic option in treating fibromyalgia pain Radiographic findings Cervical spine goes from curvature to straight (RA similar to fibromyalgia) - LOSS OF CERVICAL CURVATURE Polymyalgia Rheumatica INCREASED ESR > 50 mm/h Normochromic normocytic anemia in 50% of cases Normal creatinine Negative RF Mild liver elevations Normal (negative) muscle biopsy findings Treatment - Corticosteroids Sjogren’s syndrome Blood test abnormalities Hematology - Lymphopenia - Thrombocytopenia - Hemolytic anemia - High SED rate Immunology - Increased IgG - Low C4 Others - Abnormal liver function - Increased CK (muscle) *Treatment modalities for RA NSAIDs - Reduce the pain and swelling - Do not alter the course of the disease  - Do not use chronically; bad GI side effects Glucocorticoids - For treatment of flares - For extra-articular RA like rheumatoid vasculitis and interstitial lung disease (works good for this) - Act as bridge therapy for 6-8 wks before DMARDs begin - Use maintenance of 10 mg or less; can be used in pregnancy; HTN anxiety and (osteoporosis)? As side effects DMARDs - ALTER THE COURSE OF DISEASE METHOTREXATE ALWAYS USED EITHER ALONE OR IN COMBO (can cause hepatotoxicity) Have delayed appearance for weeks to months Biologics - Anti TNF, IL-1 and IL-6 antagonists, anti CD20 antibody, T cell co stimulatory inhibitor For monitoring treatment, go through diagnostic criteria and check off Surgical approaches Synovectomy Total joint arthroplasty and arthroscopy - Doesn’t last bc RA is a joint destroying disease - Maybe for stable pt in remission Arthrodesis