MKSAP 12 Rheumatology Board Review Cases
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MKSAP-12 RHEUMATOLOGY BOARD REVIEW INSTRUCTIONS The MKSAP-12 Board Review for Rheumatology includes: a printed copy of 70 case presentations and questions, entitled “MKSAP-12 Rheumatology” page 57-75, available from the Olive View-UCLA Medical Center Department of Medicine or Library AND this Powerpoint presentation by Dr. Philip Clements and Dr. Andrew Wong, which supplements the MKSAP-12 Rheumatology question and answer section, page 103-128. Open the powerpoint file (double click). The opening screen will show the menu bar on top. Select SLIDE SHOW on the menu bar, then choose the first command “View Show” (one click). To advance, use your left mouse button, or your DOWN arrow key. To go back, use your UP arrow key. To QUIT the presentation, hit “ESC” (escape). You will return to the opening screen, then hit Exit to quit Powerpoint. You will then return to the website.
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Case 1 Radiologic hallmarks of OA: Joint space narrowing Osteophytes Subchondral bony sclerosis Subchondral cysts PE shows marked loss of motion (cannot fully extend hip by 30o; 10o total rotation) Joint replacement is indicated when all other measures no longer control pain. Often it is pain at night that convinces the patient to have surgery.
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Case 2 Bimodal distribution of deaths in SLE: Early: SLE complications and infection Late: Accelerated atherosclerotic CV disease Pertinent findings or lack of them: Substernal chest pain in 39 y/o F with SLE Pain not positional nor pleuritic Lack of antiphospholipid antibodies T-wave changes
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Case 3 Sjogren’s is an autoimmune inflammatory disease which destroys the exocrine glands (eyes, mouth, bronchial, vaginal). It is associated with multiple autoantibodies: ANA 80% Rheumatoid factor 90% SSA / SSB 60-70% Elevated Ig levels 80% Elevated sed rate 80-90%
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Parotid gland biopsy Inflammatory lymphocytes Damaged gland
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Case 4 Sudden onset of low back pain after activity + pain radiating down right leg. Straight leg raising positive (raising leg makes pain go down the leg). Neuro intact. Dx most likely acute low back (disc) or facet joint strain with radicular pain. Rx: Pain meds, corset (maybe), encourage activity as tolerated. Imaging has high false positive rate. Image if suspect CA, osteoporotic fracture
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Case 5 Suspect limited Wegener’s (a chronic granulomatous vasculitis), which can present as an inflammatory process of larynx (producing subglottic stenosis), ears and/or eyes. Chances of finding Wegener’s (granulomatous vasculitis) by tissue bx: Upper airway bx may show inflammation, necrosis, granulomas—rarely vasculitis. Renal bx: focal GN, no Ig depositis (pauci-immune) Open lung: granulomatous inflammation AND vasculitis ANCA (esp Proteinase 3) strongly supports Wegener’s Other rheum serologies negative (ANA, RF, SSA, SSB)
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Case 6 L/S spine degenerative disease can affect the disc and facet joints. Painful syndromes can occur in soft tissues downstream, in a Christmas-like affect (paraspinal muscles, troch bursa, ant thighs)
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Case 7 Hip pain often refers to groin, buttock and anterior thigh (~ L3, L4). Acute or subacute hip pain in SLE patients on steroids should suggest possibility of AVN. MRI diagnostic procedure of choice for AVN. Disease process: Ischemia infarcts medullary bone . Cartilage and thin layer of bone fed by synovial fluid. Dead bone and cartilage eventually collapse. Then OA takes over.
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AVN Early Late
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Case 8Enteropathic arthropathies Inflammatory bowel disease Enterogenic reactive arthritis Whipple’s Celiac disease Intestinal bypass surgery
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Case 8Celiac disease Arthralgias/arthritis occur in 28% of Celiac pts. Patterns of arthritis Polyarthritis Oligoarthritis Spondylitis Onset of arthritis may precede or follow GI Sxs A gluten-free diet reverses the arthritis.
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Case 9 Hypertrophic pulmonary osteoarthropathy: Diffuse bilateral upper and lower extremity pain COPD in older smoker Clubbing Tender, painful distal ulna and radius, tibia Non-inflammatory joint fluid High sed rate Look for lung CA with chest x-ray. Document HPO with radioactive bone scan or periosteal elevation in long bone x-rays.
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Case 10 Inflammatory polyarthritis of short duration: think viral illness. Characteristics of Parvovirus B19 infection: Women who care for sick children Transient generalized rash can occur Usually small joint swelling, and pain is usually symmetrical Arthritis usually resolves in 2-4 weeks IgM parvovirus titers positive Occasionally RF and ANA may be positive evanescently Non-erosive arthritis
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Case 11 CPPD secondary to hemochromatosis: OA like changes in MCPs unusual in OA—usually means CPPD OA changes in knee may be from CPPD—supported by chondrocalcinosis (cartilage calcifications) of menisci Liver biopsy to assess iron load in hemochromatosis. Treating hemochromatosis changes patients’ lives—doesn’t help the joint disease.
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CPPD Chondrocalcinosis OA-like changes of MCP joints
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Case 12 Polyarthritis, diarrhea, pleuritic chest pain and weight loss (from malabsorption) in middle-aged male—consider Whipples in Diff Dx. Small intestine biopsy looking for PCR sequence of Tropheryma whippelii, which is essential to making the diagnosis. Lymph node biopsy looking for PAS-positive macrophages supports Dx.
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Case 13 Amyloid (?2-microglobulin) suggested by: 13 years of ESRD on hemodialysis (50% p 13 yrs). Numbness & tingling in thumb and index finger suggests CTS (median nerve distribution). Pain on motion and LOM of shoulders. Treatment of choice—renal transplant and get off hemodialysis.
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Case 14 Antiphospholipid syndrome suggested by: Blood clots 2 or more miscarriages or abortions (esp 1st trimester) 2 or 3 of these lab abnormalities for APS: Anticardiolipin Abs (IgG or IgM) Lupus anticoagulant (DRVVT) ?2-glycoprotein This patient had no lab abnormalities of APS and only one miscarriage
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Case 15 Young male with 2 DVTs (17 y/o and now at 24 y/o). Family history of DVTs. Most frequent inherited cause of DVT in white pts is Factor V Leiden deficiency. Work up for APS and other inherited causes of hypercoaguability (i.e., deficiencies of protein C or S and antithrombin 3).
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Case 16 Pt had some improvement, but is still weak and has high CPKs while on high doses of steroids—not adequately controlled. As such at high-risk of steroid AEs. Add DMARD: Methotrexate most widely use RCT showed benefit from Imuran (azathioprine)
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Case 17 Amyotrophic dermatomyositis (skin rash without muscle weakness). “Without muscle weakness there is no myositis”—paraphase from Carl Pearson, MD Dermatomyositis in older person (>60 y/o) may mean cancer—check the “usual suspects” (breast, cervix, ovaries, chest)
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Case 18 Henoch-Schonlein purpura (HSP): Antecedent “viral” infection (esp URI) Arthralgias and/or arthritis Abdominal pain frequent “Palpable purpura”, esp lower extremities (leukocytoclastic vasculitis and IgA deposits on “punch” biopsy) Some renal involvement (esp GN) common—IgA positive immunofluroescence on biopsy Usually treat conservatively. Severe GI or renal involvement may require steroids.
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Case 19Polymyalgia rheumatic Shoulder and pelvic girdle pain/stiffness, esp AM Fatigue, anorexia, mild weight loss Occasionally mild synovitis Mild anemia Over 55 years old Sed rate usually elevated—occasionally not by much Increased alkaline phosphatase in 1/3 of patients “Dramatic” response to therapeutic trial of low dose prednisone (20 mg/d), usually within 24-72 hours.
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Case 20 Transverse myelitis of spinal cord in SLE: Sudden onset of symptoms Neurologic deficit of lower extremities with urinary and/or fecal incontinence suggest cord disease: Weakness of both legs (subjective and objective) Difficulty urinating, fecal incontinence Upper and lower motor neuron findings Rectal exam shows weak anal sphincter MRI most sensitive test for finding edema of the spinal cord Check for antiphospholipid antibodies
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Case 21 This part of the clinical picture suggests Reactive Arthritis (AKA Reiter’s): 4 week hx diarrhea 3 week hx asymmetric polyarthritis, predominantly affecting lower extremities, with enthesopathy and skin lesions on bottom of feet. The 3 month hx of temp to 101o F, fatigue, 22 lb weight loss suggests something in addition. Anyone with new onset Reactive Arthritis (Reiter’s) deserves an HIV test.
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Anyone with new onset reactive arthritis deserves and needs to be tested for HIV.
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Case 22 0 1 2 3 4 5 6 5 4 3 2 1 0 Palindrome: “It leaves as it came.” After one or two weeks of arthritic complaints, the patient and her/his joints return to their previous condition—without a trace. The episodes recur and leave without a trace. Madam, I’m Adam (madam im adam)
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Hepatitis C associated syndromes Vasculitis (essential mixed cryoglobulinemia) Digit gangrene Nephritis Mononeuritis multiplex (foot drop, wrist drop) Arthritis Inflammatory polyarthritis (clinically can look like RA but is usually nonerosive) Rheumatoid factor positive
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Case 24Adult Still’s High spiking fevers Arthralgias, arthritis Maculopapular rash Lymphadenopathy Serositis Sore throat (90%), cultures negative Negative RF and ANA Leukocytosis Hepatosplenomegaly
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Case 25 CPPD Short stubby crystal Weakly positively birefringent under polarized compensated light Verboten meds Colchicine Q2h is in-humane No NSAIDs with renal insuff Allopurinol doesn’t work here Intra-articular steroid best Rx in renal or GI disease Polarized Compensated light Regular light
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Case 26 Five factors differentiate inflammatory back pain: Onset before 40 y/o Insidious onset Persistence for at least 3 months Associated morning stiffness Improvement with exercise Only 10-20% of male first-degree relatives who inherit B-27 actually develop AS.
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Lumbar strain Mechanical back pain Sudden onset (only ~half remember an inciting incident) Minimal stiffness Hurts more with exercise Gets better lying down Pain may accentuate with cough or straining Motion may be limited but Schober usually OK
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Case 27 Chemotherapeutic agents mask the inflammatory aspects of hepatitis. Hepatitis may flare when pt taken off MTX. Better in NOT to give MTX in the face of Hep C. HIV + pts may convert to full-blown AIDS if given MTX. AVOID MTX in HIV potential pts (new Reactive Arthritis) until their HIV tests are negative.
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Case 28 Polymyalgia Rheumatica Profound limb girdle stiff Anemia Elevated sed rate Very responsive to 15-20 mg prednisone QD—Rx trial Vascular symptoms may indicate concomitant temporal arteritis: Headache Jaw claudication Visual changes (amaurosis) Scalp tenderness Cough
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Case 29 1st CMC joint frequent site for OA. Tenderness on the joint. Crepitus, pain on motion of 1st CMC Extensor tendons of thumb not tender—and Finkelstein’s negative (for de Quervain’s).
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Case 30 Trochanteric bursitis is often a sign of something else (hip OA, sore feet, new shoes). Patients say “hip hurts” but point to lateral hip. Tender lateral femur, about 4-6 inches below tuberosity. Injection best choice—direct Rx without systemic toxicity. Troch bursa
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Case 31 Wait 2 wks after attack to start uricosuric agent. Benemid is first choice uricosuric agent. Allopurinol first choice if: Creatinine >2.0 mg/dl Tophi Urine uric acid >800mg per 24 hours Chemotherapy Hx of renal stones Allergy to Benemid
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Case 32 By history and exam, his RA has “burned out.” Some patients may reactivate when they become more active physically. Probably doesn’t need 7.5 mg prednisone—reduce to 5 or less mg daily, a dose which preserves bone. Pts on long term steroids need prophylactic bisphosphonates or calcitonin started when steroids are started.
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Case 33Lupus-like syndrome: Drugs implicated Common Procainamide Hydralazine Uncommon Minocycline ?-blockers D-penicillamine Isoniazid Quinidine PTU Hydantoins Trimethodione Chlorpromazine
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Case 34 Rule of thumb: No sclerodactyly No Raynaud’s/nailfold changes No ANA Equals NO SCLERODERMA! Think of some other entity, like eosinophilic fasciitis (EF). EF usually has high percent eosinophils on WBC + diff. Bx for EF requires open, deep fascial biopsy—not punch. Peau d’orange
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Case 35 Diabetic neuroarthropathy Affecting intertarsal & tarsometatarsall joints here. Marked destruction and erratic calcifications. In spite of marked erosions, may have little pain, unless infected. Sensation and proprioception usually impaired. Normal Diabetic
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Case 36 Five factors differentiate inflammatory back pain (spondyloarthropathies or SAs): Onset before 40 y/o Insidious onset Persistence for at least 3 months Associated morning stiffness Improvement with exercise NSAIDs are for pain in SAs. Patient probably has a spondyloarthropathy (undifferentiated) and needs to learn stretching exercises and posture training.
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Case 37 ELISA testing for Borrelia burgdorferi notoriously gives false positive results (3-7% normals). Needs confirmation by Western blot. Absence of IgG Ab at 3 years (in pt not Rxd with Abx) is against active or ongoing Lyme. Pt’s knee problem probably OA—Rx with acetaminophen. E. Chronicum migrans
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Case 38 RCTs have shown Omega-3 fatty acids and high salmon/halibut diets to be anti-inflammatory. Lose 8% strength per week by immobilizing. Temperature in joint increases with applied heat. Glucosamine/chondroitin are mildly anti-inflammatory and reduce the pain of OA. Acupuncture reduces pain, not inflammation.
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Case 39 SSc is associated with esophageal hypomotility, leading to dysphagia and dyspepsia. SSc pt with increasing dysphagia and wt loss needs evaluation of esophagus for stricture or obstruction (not an UGI).
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Case 40Remitting, Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE): Usually in older persons. Pitting edema of hands. Swelling reduces the ability to make a fist. May be related to polymyalgia rheumatica. Very responsive to prednisone and not to standard NSAIDs. Remember to start bisphosphonate or calcitonin to prevent bone loss.
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Case 41 Patient quotes: “I hurt all over.” “I feel like a truck hit me.” “This fatigue is the worst.” “I don’t sleep worth a damn.” d d
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Case 42 The history and physical findings suggest an ankle strain, caused by putting her weight down on her left foot in a particularly uneven area. Adequate exam and evaluation of range of motion is hindered by acute swelling. Treatment plan: Follow-up exam Apply ice Use brace to promote early ambulation
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Case 43 Migratory polyarthritis. Skin pustules. Joint effusions are often inflammatory (wbc>2.0) but usually sterile. Culture portals of entry (pharynx, anus, urethra). Culture pustule also. Discount a sexual history which is negative.
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Disseminated Gonococcal Infection In subjects <40 years old, GC is the biggest cause of joint infection. Since STDs tend to occur together, test for chlamydia, syphilis and HIV. Also Rx blind for chlamydia. Dx by clinical suspicion and culturing portals of entry (throat, anus and urethra). Best culture is directly onto Thayer-Martin medium at bedside.
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Case 44 Ruptured Baker’s Cysts Crescent sign
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Pseudo-phlebitis of ruptured popliteal (Baker’s) cyst Some signs of ruptured cyst may resemble phlebitis:Abrupt onset of calf swelling, tenderness, positive Homan’s sign. Other characteristics of ruptured cyst differ: Knee effusions common. Best lab test is ultrasonography—looking for Baker’s cyst and/or phlebitis of calf and thigh. Best Rx is steroid injection into suprapatellar bursa or medial knee joint—knee hydraulics will pump it into posterior knee.
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Case 45 Pulmonary hypertension: occurs in 30-40% of SSc on echo. severe PHT seen in 10%. often as isolated PHT without significant ILD. Isolated PHT occurs predominantly in limited SSc of long duration. Low DLCO is the clue (often <50-55% predicted). Prominent Pulmonary Artery
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Case 46 Synovial fluid essential for Rx of acute inflammatory synovitis: Hemarthrosis Crystal-induced Septic arthritis Reactive arthritis Traumatic arthritis Overuse from traveling When INR in Rx range (2-3), it is safe to do arthrocentesis.
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Case 47 X-ray shows flattening of head and joint incongruity —later it will show OA. 2-5% of alcoholics affected. Steroids other major risk—especially in SLE or vasculitis. If suspect AVN and plain x-ray normal, get MRI—best technique for showing early AVN.
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Case 48 In the NIH trials, the probability of avoiding renal failure at 10-12 years in SLE DPGN was: 90% with cyclophosphamide treatment 60% with azathioprine (this was not significantly different than cyclophosphamide only 20% with prednisone
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CTX Rx of SLE DPGN Short course: 6 monthly infusions Long course: 6 monthly infusions, followed by quarterly infusions for 2 more years.
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Case 49 Lumbar Stenosis
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Tip-offs to lumbar stenosis Body maneuvers which extend the L/S spine narrow the canal (and compress the nerve roots), while spine flexion opens the canal). Increases symptoms: Walking (pseudoclaudication*) Walking down hill Leaning backwards Lying prone in bed * Pulses should be intact and ankle-arm index ? 1.0 Decreases symptoms: Sitting still Bending forward while walking (shopping cart) Walking with cane Walking uphill Lying supine in bed
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Case 50 Cheiroarthropathy: “Sclerodactyly” No Raynaud’s Capillary loops normal ANA negative Flexion contractures of fingers give “prayer sign” Usually type I Diabetes
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Diabetes mellitus Other MSK complications of diabetes: Dupuytren’s Trigger fingers Adhesive capsulitis (“frozen”) of shoulder
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Case 51 Enzyme profile in PM (with or without SLE) CPK 95% Aldolase most cases ALT most cases AST most cases LDH most cases
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Case 52 In RF + patient with inflammatory polyarthritis of the rheumatoid type (RA-like), check for Hep C first. If Hep C test is negative, can use MTX. Therapeutic progression (first year of RA)-- keep adding therapies until RA controlled: NSAID and/or low dose prednisone. Add hydroxychloroquine and/or sulfasalazine. Add methotrexate (7.5 to 25 mg weekly) with folic acid QD. If max doses of MTX don’t control, add anti-TNF-? inhibitor or leflunomide.
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Case 53 Scleroderma + ?BP + ? Creatinine = Renal * Crisis * Until proven otherwise
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Case 54 Two FDA-approved saliva stimulators Pilocarpine (Salagen) 5 mg qid Cevimeline (Evoxac) 30 mg tid
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Case 55 Hypothyroidism Carpal tunnel syndrome Non-inflammatory synovial fluid Elevated CPKs Fibromyalgia Numb areas in CTS
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Case 56 Ig + proliferative GN Proteinuria (>500 mg/d) Often pedal edema RBCs and WBCs in RUA ANA + Often anti-dsDNA Ab + Normal-low C3, C4 Creatinine nl or up
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Case 57 Mild osteoarthritis Crepitus = OA Good exercises: Walking Isometric quad strengthening Consider acetaminophen also Normal knee x-ray
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Case 58Lupus-like syndrome: Drugs implicated Common Procainamide Hydralazine Uncommon Minocycline ?-blockers D-penicillamine Isoniazid Quinidine PTU Hydantoins Trimethodione Chlorpromazine
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Case 60 Total protein – albumin = globulin (which in this case is elevated). Anemic High ESR, CRP, SAA, ?-glob, fibrinogen, haptoglobin, etc. Dx: multiple myeloma ESR in female = Age + 10 2 D
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Case 61 On too much steroid! Hydroxychloroquine is good for SLE rash and arthritis. SLE arthritis is usually NON-erosive.
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PolychondritisEpisodic inflammation of hyaline cartilage Ears and/or nose common Larynx and tracheal cartilage most life-threatening Non-erosive arthritis Eyes (scleritis) Aortic regurgitation Panniculitis of skin
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Case 63 Antiphospholipid syndrome suggested by: Blood clots 2 or more miscarriages or abortions 2 or 3 of these lab abnormalities for APS: Anticardiolipin Abs (IgG or IgM)—moderate to high level Lupus anticoagulant (DRVVT) ?2-glycoprotein Lupus anticoagulant cannot be assessed when the patient is being treated with heparin—warfarin yes.
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Case 64 “Chondrocalcinosis” on x-ray suggests CPPD. OA-like changes of 2nd and 3rd MCP suggest CPPD. Diff dx of CPPD includes: Hemochromatosis Hyperparathyroidism Acromegaly Diabetes mellitus Rx of 2 diagnoses changes lives: Hemochomatosis (Fe, transferrin/ IBC, ferritin) Hyperparathryoidism (Ca, Ph, PTH)
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Case 65 Reflex Sympathetic Dystrophy (RSD): Follows injury (sometimes minor)—usually extremity. Constant dysesthesia, often disproportionate to injury. Vasomotor changes are frequent: Early: hyperemia and swelling of skin, exquisite tenderness Late: cold, hyperhidrotic skin; atrophy of skin and muscle; contractures; osteopenia Lab and path studies usually negative. Three-phase radioactive bone scan often positive.
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Case 66 APS often affects veins and arteries. TIAs and strokes in SLE are often emoblic from valve vegetations. Mitral valve vegetations
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Case 67 Differential dx of E.Nodosum: TB, Fungal Strept, Yersinia Birth control pills, Sulfa Behcet’s, IBD Sarcoid
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