Rheumatology Board Review Part 1 “Just the Pearls Please” Gerald Falasca, M.D. Medical Specialists of Johnson City April, 2015 Disclosure Statement of Financial Interest I, Gerald Falasca, DO NOT have a financial interest/arrangement or affiliation with one or more organizations that could be perceived as a real or apparent conflict of interest in the context of the subject of this presentation. Disclosure Statement of Unapproved/Investigative Use I, Gerald Falasca, DO NOT anticipate discussing the unapproved/investigative use of a commercial product/device during this presentation. Rheumatoid Arthritis • Which patient is most likely to have rheumatoid arthritis? A. 35 year old female with pain and swelling of wrists & MCPs, two hours of morning stiffness, carpal tunnel syndrome, and negative rheumatoid factor (RF). B. 30 year old male with pain and swelling of wrists, MCPs, and knees, one hour of morning stiffness, diffuse rash in the afternoon, pharyngitis, leukocytosis, negative rheumatoid factor (RF). C. 20 year old male with chronic low back pain, swollen left knee, chronic uveitis, negative RF. Rheumatoid Arthritis • Which patient is most likely to have rheumatoid arthritis? A. 35 year old female with pain and swelling of wrists & MCPs, two hours of morning stiffness, carpal tunnel syndrome, and negative rheumatoid factor (RF). B. 30 year old male with pain and swelling of wrists, MCPs, and knees, one hour of morning stiffness, diffuse rash in the afternoon, pharyngitis, leukocytosis, negative rheumatoid factor (RF). C. 20 year old male with chronic low back pain, swollen left knee, chronic uveitis, negative RF. Rheumatoid Arthritis • • • • • • Symmetric, small joint polyarthritis Morning stiffness >1 hour Hand and wrist involvement Fatigue Greater than six weeks Radiographs: Erosions, periarticular osteopenia, joint space narrowing Early Signs of Aggressive RA • • • • • • • >20 joints involved High titer RF or anti-CCP Anemia; smoking High sed rate or CRP Early erosions Extra-articular features High multi-biomarker disease activity score* *Ann Rheum Dis. 2014 May 8. Hambardzumyan K et al. doi: 10.1136/annrheumdis-2013-204986. [Epub ahead of print] • Which genetic marker is most strongly associated with seropositive (RF positive), aggressive rheumatoid arthritis in Caucasians? • HLA-B27 • HLA-DR4 • PTPN22 (protein tyrosine phosphatase N22) • Which genetic marker is most strongly associated with seropositive (RF positive), aggressive rheumatoid arthritis in Caucasians? • HLA-B27 • HLA-DR4 • PTPN22 (protein tyrosine phosphatase N22) • A. B. C. D. E. Which of the following treatments for rheumatoid arthritis could be used during pregnancy if necessary (may choose more than one)? Prednisone Etanercept Methotrexate Leflunomide Hydroxychloroquine • A. B. C. D. E. Which of the following treatments for rheumatoid arthritis could be used during pregnancy if necessary (may choose more than one)? Prednisone Etanercept Methotrexate Leflunomide Hydroxychloroquine • How would you treat this patient (she had hysterectomy)? 35 year old female with pain and swelling of wrists & MCPs, two hours of morning stiffness, carpal tunnel syndrome, and negative rheumatoid factor (RF): A. B. C. D. Methotrexate 15 mg once a week Hydroxychloroquine 200 mg BID Sulfasalazine 1000 mg BID Etanercept 50 mg SC once a week • How would you treat this patient (she had hysterectomy)? 35 year old female with pain and swelling of wrists & MCPs, two hours of morning stiffness, carpal tunnel syndrome, and negative rheumatoid factor (RF): A. B. C. D. Methotrexate 15 mg once a week Hydroxychloroquine 200 mg BID Sulfasalazine 1000 mg BID Etanercept 50 mg SC once a week Swan neck deformities and MCP synovitis in patient with RA/SLE overlap syndrome. MTP erosions and joint space narrowing in RA. Rheumatoid Arthritis • 75-80% will have positive rheumatoid factor. • Anti-CCP is slightly less sensitive, considerably more specific. • CCP = cyclic citrullinated peptides Management Tips • Often improves during pregnancy. • Neck pain or neurological symptoms suggests need for C1-C2 flexion/extension views, espec preoperatively. • Acceptable during pregnancy: prednisone, hydroxychloroquine, sulfasalazine, TNF inhibitors. Rheumatoid Arthritis • Flareup in a single joint? – Always tap monoarthritis on the boards (to rule out septic arthritis). Sjogren syndrome • Dry eyes (gritty sensation) • Dry mouth • Sometimes, parotid gland enlargement. • Gold standard: minor salivary gland biopsy. • Silver standard: salivary flow scan Rheumatoid Lung & Heart • Pleural effusions in RA are exudative and have very low glucose ( < 20 mg/dl). If culture negative, treat with steroids, not chest tube. • Caplan’s syndrome (rheum nodules in lung occurring in pt with RA and pneumoconiosis). Usually need bx. • Pericarditis/pleuritis Caplan’s Synd. Felty’s Syndrome • RA plus neutropenia plus splenomegaly +/- infections +/- leg ulcers. • Treat infections; sometimes use GCSF Rheumatoid Treatment • NSAIDs do not slow progression of disease; only the DMARDs do that. • Treatment for the non-pregnant patient begins with methotrexate. • May add low-dose prednisone early on. • If inadequate response, or if erosions/joint damage progress, add a TNF inhibitor usually. Extensor tendon rupture from RA; (Can’t extend 4th, 5th fingers) • A 55 year old male with a 10 year history of rheumatoid arthritis presents with a one week history of right swollen and moderately painful calf as shown. No pulmonary symptoms. You send patient for lower extremity ultrasound for DVT which is negative. Most likely diagnosis is: A. B. C. D. DVT missed by ultrasound Lymphedema Uncontrolled rheumatoid synovitis of knee Bakers cyst • A 55 year old male with a 10 year history of rheumatoid arthritis presents with a one week history of right swollen and moderately painful calf as shown. No pulmonary symptoms. You send patient for lower extremity ultrasound for DVT which is negative. Most likely diagnosis is: A. B. C. D. DVT missed by ultrasound Lymphedema Uncontrolled rheumatoid synovitis of knee Bakers cyst Baker’s cyst in calf Sjogren’s Syndrome • Symptoms: dry eyes (presents as gritty sensation) and dry mouth, sometimes parotid gland enlargement • Two kinds: secondary (associated with another connective tissue disease, most commonly rheumatoid arthritis) and primary Sjogren's. • Hepatitis C can mimic Sjogren's, including histology. Suspect it when there are liver abnormalities and/or cryoglobulinemia. • HIV infection can also mimic Sjogren's, complete with lymphocytic infiltrates. Sjogren’s Syndrome • Other causes of parotid gland enlargement are diabetes, sarcoid, ethanol abuse, amyloid, lymphoma. • Most common causes of dry eyes/mouth are "senile atrophy" and medications. Sjogren’s Syndrome • 20-30% of secondary and 70% of primary forms will have +SSA antibodies. HIV type does not have SSA. • Important association of SSA antibodies on the boards is with congenital heart block in babies whose mothers (many of whom have lupus) carry SSA. Risk is 2% of CHB if mom carries SSA. Risk in recurrent pregnancies is 15%. Sjogren’s Syndrome • Primary SS is a systemic connective tissue disease similar to lupus. May have fever, diffuse adenopathy, peripheral neuropathy, pulmonary symptoms, CNS symptoms, polyclonal gammopathy, leukopenia, pancreatic dysfunction, renal abnormalities. Sjogren’s Syndrome • Definitive diagnosis is by minor salivary gland biopsy (shows clumps of lymphocytes). In the 1° and 2° forms the infiltrating cells are mostly CD4. In the HIV type the cells are CD8, since HIV patients are out of CD4 cells. • A screening test for dry eyes is the Schirmer test; it does not diagnose Sjogren's. • Another useful approach is the Sjogren scan (salivary flow scan) • Treatment is symptomatic, also hydroxychloroquine. Still’s Disease • Fever • Evanescent, salmon colored, MP rash, especially late afternoon • Leukocytosis • Elevated ferritin (in the 1000’s) • Polyarthritis Lupus • • • 11 criteria (malar rash, discoid lesions, photosensitivity, oral ulcers (hard palate usually), arthritis, serositis, renal disorder (proteinuria or casts), neurological disorder (seizure or psychosis), hematologic disorder (hemolytic anemia (not any old anemia), leukopenia or thrombocytopenia), immunologic disorder (anti-DNA, antiSmith, false positive RPR, positive LE prep), positive ANA) . Diagnosis should include 4 or more criteria. Note that alopecia, while common in lupus, is not an official criterion for diagnosis. Anti-cardiolipin antibody or a lupus anticoagulant is as good as a false positive RPR). Lupus • ANA (on the boards) will almost always be positive, moderate or high titer (>1:320), usually homogenous or rim (peripheral) pattern. • The combination (on the boards) of low C3, low C4, +ANA, +DNA is highly suggestive of dx of SLE. Lupus Nephritis Which patient does not need kidney biopsy right now? • 20 y.o. female with polyarthritis, malar rash, photosensitivity, +anti-DNA, +SSA, trace proteinuria, trace hematuria, low C3, low C4. • 20 y.o female with polyarthritis, malar rash, +anti-DNA, +Smith, 2+ proteinuria, red cell casts. • 20 y.o. female with polyarthritis, malar rash, +anti-DNA, low C3, low C4, 3+ proteinura. Lupus Nephritis Which patient does not need kidney biopsy right now? • 20 y.o. female with polyarthritis, malar rash, photosensitivity, +anti-DNA, +SSA, trace proteinuria, trace hematuria, low C3, low C4. • 20 y.o female with polyarthritis, malar rash, +anti-DNA, +Smith, 2+ proteinuria, red cell casts. • 20 y.o. female with polyarthritis, malar rash, +anti-DNA, low C3, low C4, 3+ proteinura. Lupus • Use C3, C4, DNA titer to follow lupus activity; they improve. anti-Smith does not change with time (usually). • Most persons with isolated discoid lupus do not get SLE. • P-ANCA are common in SLE. • A. B. C. D. E. Which of the following antibodies is most specific for SLE? Anti-double stranded DNA Anti-single stranded DNA Anti-Smith SSA Anti-ribosomal P • A. B. C. D. E. Which of the following antibodies is most specific for SLE? Anti-double stranded DNA Anti-single stranded DNA Anti-Smith (also most specific for renal involvement) SSA Anti-ribosomal P Lupus • Use antimalarials for arthritis, skin and as a steroid-sparing agent (nearly all lupus patients should get an antimalarial). • Use steroids for more serious manifestations. • Do kidney biopsy if patient has cellular casts, hematuria + proteinuria, proteinuria > 500 mg/d, or elevated creatinine (next slide). • Treat diffuse proliferative GN and membranous GN with prednisone and monthly IV cyclophosphamide or with mycophenolate mofetil (CellCept). • Serious CNS lupus: Tx with cyclophosphamide. Indications for Initial Kidney Biopsy in SLE • Protein excretion > 500 mg/d OR • Active sediment (>=5 RBCs/HPF, most of which will be dysmorphic, and cellular casts) Indications for REPEAT Kidney Biopsy in SLE • Active sediment in previously quiescent disease. • Unexplained rise in creatinine. • Worsening proteinuria despite tx. Treatment of Diffuse Proliferative Lupus Nephritis ACEI or ARB with BP <= 130/80 Statin therapy Hydroxychloroquine Induction therapy with either IV cyclophosphamide or with mycophenolate mofetil. • Prednisone 0.5 – 1 mg/kg/d x 2 weeks, then tapered over months. • Pulse steroids (500 – 1000 mg/d) x 3 days for severe nephritis. • • • • Lupus Pregnancy • Your 22 y.o. lupus patient is 2 months into her treatment for DPGN with MMF and prednsione. Urinary sediment is still active. She finds herself pregnant despite your warnings to the contrary. Of the following options, which is optimal? • D/C MMF, do not replace. • D/C MMF, increase pred from 20 mg/d to 40 mg/d. • Switch MMF to azathioprine. • Switch MMF to cyclosphosphamide. Lupus Pregnancy • Your 22 y.o. lupus patient is 2 months into her treatment for DPGN with MMF and prednsione. Urinary sediment is still active. She finds herself pregnant despite your warnings to the contrary. Of the following options, which is optimal? • D/C MMF, do not replace. • D/C MMF, increase pred from 20 mg/d to 40 mg/d. • Switch MMF to azathioprine. • Switch MMF to cyclosphosphamide. Which factor carries the worst prognosis for renal survival at 5 years in lupus nephritis? A. Blood pressure B. Age C. Chronicity index D. Activity Index E. Black race Cyclophosphamide therapy for lupus nephritis: Poor renal survival in black Americans. Glomerular Disease Collaborative Network. Dooley MA, Hogan S, Jennette C, Falk R Kidney Int. 1997;51(4):1188. Which factor carries the worst prognosis for renal survival at 5 years in lupus nephritis? A. Blood pressure B. Age C. Chronicity index D. Activity Index E. Black race Cyclophosphamide therapy for lupus nephritis: Poor renal survival in black Americans. Glomerular Disease Collaborative Network. Dooley MA, Hogan S, Jennette C, Falk R Kidney Int. 1997;51(4):1188. Lupus • The rash of subacute cutaneous lupus can mimic disseminated Lyme disease, psoriasis. • Anti-Smith correlates with serious kidney involvement. Subacute cutaneous lupus (left). Oral ulcers (above). Discoid lupus Lupus Not Lupus (but what?) Livedo reticularis Digital infarcts (antiphospholipid syndrome) Alopecia (scalp) and healed discoid lesions (face) CNS lupus Drug-Induced Lupus • Fever, serositis, arthritis, rash • Hardly ever brain or kidney involvement • Classically: anti-histone antibodies. • TNFI: anti-DNA antibodies DIL: The Big Four Culprits • Procainamide (anti-histone) • Hydralazine (anti-histone, ANCA, anti-DNA) • Minocycline (ANCA) • TNF inhibitors (anti-DNA) • Many others sometimes cause DIL. Anti Phospholipid • “Clinical Finding” + Two positive tests 12 weeks apart – Medium to high titer anticardiolipin Ab – Anti β2 glycoprotein I Ab – Lupus anticoagulant (hexagonal phase phospholipid assay, DRVVT, kaolin clotting time. • Arterial or venous thrombosis Antiphospholipid Syndrome • An acquired hypercoagulable state. • Presentation with arterial or venous thrombosis, pregnancy loss, or thrombocytopenia. • About 50% have systemic lupus erythematosus. • Antiphospholipid antibodies include the lupus anticoagulant, anticardiolipin antibody, and anti-beta 2 glycoprotein-1. Antiphopholipid • A 25 year old patient with SLE has moderate titer IgG anticardiolipin antibodies. She has never had a thrombotic episode or a miscarriage. She is 8 weeks pregnant (P1G0). CBC is normal. What do you recommend? A. B. C. D. No additional medication Low dose aspirin (81 mg) Low molecular weight heparin Warfarin Antiphopholipid • A 25 year old patient with SLE has moderate titer IgG anticardiolipin antibodies. She has never had a thrombotic episode or a miscarriage. She is 8 weeks pregnant (P1G0). CBC is normal. What do you recommend? A. B. C. D. No additional medication Low dose aspirin (81 mg) Low molecular weight heparin Warfarin Management • For events: • Heparin or LMWH, then warfarin with INR 2-3 • Asymptomatic: ASA • Catastrophic: Heparin, corticosteroids, plasma exchange ± IVIG Pregnant with APA Syndrome • No previous miscarriages or thromboses: ASA only • Previous miscarriage: LMWH therapeutic anti-factor Xa levels of 0.6 to 1.0 U/mL four hours after drug administration ± ASA Scleroderma • • • A 50-year-old man with a 1-year history of diffuse cutaneous systemic sclerosis is hospitalized for new-onset hypertension with anemia and thrombocytopenia. Vitals are unremarkable except BP is 180/110 mmHg. There was sclerodactyly and skin thickening elsewhere. Neurologic examination was normal. Cardiac examination revealed a normal S1, S2, and S4, and no S3. Abdominal examination was unremarkable. There was 1+ edema of both lower extremities. HgB 9.8 g/dL with schistocytes. Platelets 100K. Creatinine 1.4 mg/dl. UA 1+ protein and small blood. Best treatment: A. B. C. D. Captopril Metoprolol Clonidine Furosemide Scleroderma • • • A 50-year-old man with a 1-year history of diffuse cutaneous systemic sclerosis is hospitalized for new-onset hypertension with anemia and thrombocytopenia. Vitals are unremarkable except BP is 180/110 mmHg. There was sclerodactyly and skin thickening elsewhere. Neurologic examination was normal. Cardiac examination revealed a normal S1, S2, and S4, and no S3. Abdominal examination was unremarkable. There was 1+ edema of both lower extremities. HgB 9.8 g/dL with schistocytes. Platelets 100K. Creatinine 1.4 mg/dl. UA 1+ protein and small blood. Best treatment: A. B. C. D. Captopril Metoprolol Clonidine Furosemide Scleroderma • Two major forms: diffuse (poorer prognosis) and limited (CREST). • CREST = calcinosis, Raynaud's, esophageal dysmotility (solids get stuck), sclerodactyly, telangiectasias. • Both forms present with Raynaud's (correct sequence of colors in Raynaud's is white, blue, red). Scleroderma • In either form the presenting complaint is often either Raynaud's or puffy hands (not sclerosed hands). Puffy hands precede sclerosis. • Telangiectasias are often found on face or palms in CREST. Scleroderma • Pulmonary hypertension is now treatable with bosentan, an oral endothelin antagonist ($50,000 per year), iloprost (inhaled) (costs more), treprostinil (IV, SC), epoprostenol (IV), sildenafil (oral). Scleroderma • Count on seeing scleroderma renal crisis on boards. • Schistocytes on peripheral smear signal renal crisis. • Use ACE inhibitor to control scleroderma renal crisis, even if patient has elevated creatinine. Push dose of ACEI to max to control BP. Courtesy of Amy Evangelisto, M.D. Courtesy of Amy Evangelisto, M.D. Courtesy of Dr. Amy Evangelisto Rheumatic Fever • In adults the arthritis is generally large joint, asymmetric, lower extremity, additive, with pain out of proportion to objective inflammation. • ASO titer usually quite high. • Note that ASO titer (or the screening streptozyme test) commonly positive in normal persons since it merely indicates a recent strep infection. Rheumatic Fever • Carditis less common in adults. • Jones criteria: – Major: arthritis, carditis (abnormal EKG alone usually doesn't count), chorea, nodules, erythema marginatum; – Minor: fever, arthralgia, high ESR/CRP. • Need two major, or one major with two minor with evidence of preceding strep infection (throat culture or high/rising ASO titer). Psoriatic Arthritis • Five types (these are important): 1. Oligoarticular (most common), 2. Polyarticular (mimics rheumatoid arthritis but RF is negative), 3. DIP type 4. Arthritis mutilans (rare; causes opera glass fingers). 5. Spondylitis Psoriatic Arthritis • Must know: – sausage digits – nail pits – onychodystrophy. • Patient may be unaware of presence of psoriasis. • Family history of psoriasis common. Psoriatic Arthritis • Tx serious psoriatic arthritis with TNF inhibitors on boards. – The TNFIs retard joint damage. • NSAIDS, sulfasalazine or methotrexate. • (Not FDA approved for psoriatic, and not as effective as in RA) The Clue • • A 25 year old woman comes in with fever of 100°F, diffuse urticarial rash, anorexia, nausea, and small joint polyarthritis of 3 days duration. CBC is unremarkable. ALT is 350. AST is 250. Most likely dx is: A. B. C. D. E. Acetaminophen OD Hepatitis B Hepatitis C Parvovirus Acute systemic lupus The Clue • • A 25 year old woman comes in with fever of 100°F, diffuse urticarial rash, anorexia, nausea, and small joint polyarthritis of 3 days duration. CBC is unremarkable. ALT is 350. AST is 250. Most likely dx is: A. B. C. D. E. Acetaminophen OD Hepatitis B Hepatitis C Parvovirus Acute systemic lupus Hepatitis B • Fever, anorexia, urticarial or vasculitic rash with small-joint polyarthritis. • Transaminases should be elevated at this time (the clue!). • Order HBsAg (not HBsAb) to confirm (but occasionally negative). • Note that patient is not yet jaundiced at time of arthritis! Papular acrodermatitis of childhood (GianottiCrosti disease) can be casued by several viruses including hepatitis B) Hepatitis C • Cryoglobulinemia, mixed. Usually asymptomatic in hep C ; see low C4 (as in lupus). Hep C commonly causes positive ANA (can be quite misleading). • Cryoglobulinemic vasculitis is much less common; see peripheral ischemia (digits, ears). Dx is by biopsy with positive serum cryoglobulins. • Associated with oligoarticular, mildly inflammatory arthritis too (note difference from rheumatoid arthritis) • Transaminases can be normal, yet have circulating virus. Hepatitis C • Associated with Sjogren-like syndrome with parotid gland lymphocytic infiltration. • Some may be SSA positive. Hepatitis C – Extrahepatic Manifestations • Cryoglobulins • Cryoglobulinemic vasculitis • Autoantibodies: 45-60% – RF, ANA, anti-thyroid, anti-smooth muscle, anti-cardiolipin – Usually low titer – Not clnically important • Porphyria cutanea tarda • Psoriasis-like lesions Hepatitis C – Extrahepatic Manifestations • Hypothyroidism 13% • Flares of autoimmune disease with interferon treatment. • Myasthenia gravis, sarcoid – case reports • Corneal ulcer (Mooren’s ulcer), scleritis, Sjogren’s • Membranoproliferative (usually with LCV), membranous nephritis Leukocytoclastic vasculitis with nuclear dust Parvovirus Arthritis A board exam favorite. • Classic patient is young woman who works with children. • Caused by parvovirus B-19 which also causes fifth disease, aplastic crisis in sicklers, other things. • Fever followed by diffuse maculopapular rash followed by small joint polyarthritis, sometimes with AM stiffness. • Self-limited in 4 - 8 weeks (usually). • Confirm diagnosis by ordering IgM parvovirus titer (IgG titer not helpful since indicates old infection). Reiter’s • Classic triad is arthritis (large joint, asymmetric, oligoarticular), urethritis, conjunctivitis (latter may be quite mild and transient). • Urethritis usually due to chlamydia infection, but will be sterile urethritis if Reiter's is due to enteropathic infection (always culture it). • When due to chlamydia, Reiter's frequently has associated keratoderma blennorrhagicum and balanitis circinata. Reiter’s • Treating infection is good to do but classically doesn't hasten resolution of Reiter's (though this is controversial). • Relapses and chronic smoldering course are common in this disease. • Treat initially with NSAID. • Only a minority develop spondylitis or sacroiliitis, and this may take months/years to show up. Keratoderma blennorrhagicum Ankylosing Spondylitis • Classic patient will be a young (teens, twenties) male with nagging low back pain that improves with exercise. Sed rate usually increased. Order sacroiliac films or MRI (most sensitive) to confirm diagnosis. • If fever present, rule out infection (osteomyelitis) first. • 90% of patients will be HLA-B27 positive. Bone marrow edema surrounding left sacroiliac joint on MRI (T2 weighted). Irregular sacroiliac joints on plain films. Ankylosing Spondylitis • Mild disease can be treated with NSAIDs. • More serious disease is treated with a TNFI. • Anterior uveitis is fairly common. • Aortic valve disease also associated with B27. Lyme • Look for the big things like erythema chronicum migrans (ECM), monarthritis mimicking septic arthritis, Bell's palsy, heart block. • An early Lyme presentation (fever, headache, mylagias, arthralgias – but with low WBC and elevated transaminases suggests Erlichiosis. (Also responds to doxycycline). • Fever, headache, myalgias and a rash in the summer should always suggest Rocky Mtn spotted fever (even w/o the rash). Lyme • • • • • Remember that Lyme titer is frequently negative during the ECM phase; just treat ECM presumptively. Lyme titer may stay negative or may turn positive after successful Tx of ECM. Always base treatment on Western blot, not on serology. Pearl: Do a Lyme titer for any "culture negative" septic arthritis and for most cases of Bell's palsy. Don't treat fibromyalgia patient with borderline Lyme titer for Lyme (won't get better). Confirm all borderline titers with a Western blot. If WB negative, it's a false positive. Treat most late manifestations with 3-4 weeks of doxycycline. Exception: CNS disease. Also, may wish to treat arthritis initially with IV ceftriaxone. Sarcoid • Lofgren's syndrome: bilateral ankle periarthritis, fever, erythema nodosum. To confirm diagnosis, order chest radiograph. Most cases resolve spontaneously; avoid steroids. • Treatment is NSAID initially (such as indomethacin), not steroids initially. Use steroids only for resistant cases. • Steroids may increase the conversion rate to chronic sarcoid. Lofgren’s syndrome (note the periarticular distribution of the erythema nodosum) Gonorrhea • There are two distinct rheumatologic presentations: a) migratory tenosynovitis with skin lesions, and b) septic arthritis, usually monoarticular. • Much more common in women; classically disseminates during or just after menses. What’s this? Gout • • • • • Pearl: wrist inflammation occurring acutely in an elderly, hospitalized female is usually pseudogout, but always tap to rule out septic arthritis (on the boards) Remember that crystal arthritis (especially when polyarticular) commonly causes fever, sometimes high. Allopurinol is not a treatment for acute attack of gout (may make acute attack worse). Treat acute attack with colchicine, NSAID, intra-articular corticosteroid or systemic corticosteroids. Make sure cultures are negative before injecting joint (on boards). Probenecid is hypouricemic drug of choice (when one is needed). Use allopurinol if pt is overproducer, has hx stones, hx renal insufficiency or tophi. • You are asked to see an 80 y.o. lady admitted to the hospital for pneumonia 4 days ago. She has a painful swollen knee with no history of a fall. Radiograph is shown on next slide. The pneumonia is improving and she was looking forward to being discharged on oral antibiotics. She mentioned that she had bumped her knee on the dresser two days ago. • T99.9F, WBC 12,000, Uric acid 4.8 mg/dl • Synovial fluid WBC 20,000 with 90% polys. Gram stain negative. No crystals were seen. • Most likely diagnosis is: A. Septic arthritis, same organism as pneumonia. B. Septic arthritis, different or resistant organism. C. Gout D. Pseudogout E. Lyme disease • Most likely diagnosis is: A. Septic arthritis, same organism as pneumonia. B. Septic arthritis, different or resistant organism. C. Gout D. Pseudogout E. Lyme disease • Best treatment is to: A. Aspirate, then inject with depot corticostreroid. B. Give prednisone taper. C. Change antibiotics. D. Rest, ice, elevation. E. Arthroscopic drainage. • Best treatment is to: A. Aspirate, then inject with depot corticostreroid. B. Give prednisone taper. C. Change antibiotics. D. Rest, ice, elevation. E. Arthroscopic drainage. Clinical Associations with Pseudogout • Aging • Previous joint surgery • Previous joint trauma • Familial types • Gout • Amyloidosis Hyperpara Hemochromatosis Hypomagnesemia Familial hypocalciuric hypercalcemia • Hypophosphatasia • Wilson’s disease • Ochronosis • • • • Clinical Associations with Pseudogout • Aging • Previous joint surgery • Previous joint trauma • Familial types • Gout • Amyloidosis Hyperpara Hemochromatosis Hypomagnesemia Familial hypocalciuric hypercalcemia • Hypophosphatasia • Wilson’s disease • Ochronosis • • • • Rheumatology Board Review Part 2 “Just the Pearls Please” Gerald Falasca, M.D. Medical Specialists of Johnson City April, 2015 Polymyalgia Rheumatica • See accompanying table (last page) to know how to differentiate PMR from polymyositis and from fibromyalgia. Key points below. • PMR presents with diffuse myalgias and (less commonly) arthralgia in the elderly. Often wgt loss, fever. Muscle weakness is not a feature. On the boards, sed rate will be very high ( ~ 100 mm/hr). • 10 - 15% of patients develop temporal arteritis. So, you should have low threshold to do biopsy. • Treatment is low dose steroids (10 - 20 mg/d prednisone), unless patient has temporal arteritis. Dermatomyositis • Risk of malignancy is substantial in older patients (especially > 65). • Colon cancer especially common Inclusion Body Myositis • Uncommon (except on boards). • Presents just like polymyositis, but the clues are presence of distal weakness in addition to prox. weakness, and lack of response to steroids. More atrophy. • M>F. CK < 10x ULN. • Light microscopy shows typical intranuclear or intracytoplasmic inclusions. Dx confirmed by electron microscopy. • There’s no good treatment. POLYMYOSITIS/DERMATO-MYOSITIS POLYMYALGIA RHEUMATICA Proximal muscle weakness (relatively painless). Often fever, arthralgias, weight loss, fatigue. Proximal muscle pain (shoulders, hips) with little or no true weakness. Often fever, weight loss, fatigue. Generalized pain, fatigue, "nonrestorative" sleep; the "fibrositic personality" DEMOGRAPHICS Middle age, F slightly > M Age >50, 2F:1M Young adult or middle aged females PHYSICAL EXAM Proximal muscle weakness (ie., can't get out of chair). Gottron's papules, heliotrope and periorbital edema in dermatomyositis. Proximal muscle tenderness; strength normal Characteristic "tender points" (see figure) SED RATE Elevated Elevated > 40-50 mm/hr; often >100 mm/hr Normal CPK Elevated; often in the thousands; rarely can be normal. Normal Normal TREATMENT High dose prednisone (40 - 80 mg/day) Low dose prednisone (5 - 15 mg/d) Patient education; low dose amitriptyline (10-50 mg HS); duloxetine; pregabalin; milnacipran; cyclobenzaprine ASSOCIATED DISORDERS Malignancy in 10 - 15% of adults (up to 50% if >65 yrs). Increased in polymyositis, but less so. Other connective tissue diseases such as SLE, scleroderma. Temporal arteritis in 10 - 15% Irritable bowel, headaches, dysmenorrhea, irritable bladder, depression. SYMPTOMS FIBROMYALGIA Osteoporosis Tx NSAIDS • Risk factors for NSAID-induced ulcers are advancing age, debilitating disease, CHF, prior history of ulcers, coagulopathy (or warfarin). • All NSAIDS cause stomach ulcers, but some cause fewer ulcers (i.e., non-acetylated salicylates, nabumetone, celecoxib, meloxicam, etc). • The risk of superficial erosions is approximately 20% after 1 - 4 weeks with traditional NSAIDS • Risk of serious complications (significant bleeding or perforation) is only 2% - 4% per year (higher with risk factors above). NSAIDSs • Only aspirin irreversibly inhibits platelet function (i.e., a single 80 mg baby aspirin inhibits platelet function for 7 - 10 days). • Some NSAIDS (i.e., ibuprofen) reversibly inhibit platelet function • Some NSAIDs do not inhibit platelet function: non-acetylated salicylates, nabumetone (Relafen), meloxicam (Mobic), celecoxib (Celebrex) • Ibuprofen (but not other NSAIDs) NEGATES the anti-platelet effect of ASA if given before the ASA – Give the ASA first, wait 1-2 hours, then give ibuprofen. NSAIDs • Don't give NSAIDS on the boards to persons with renal insufficiency or with recent CHF. • Don't give NSAIDS on the boards to persons with history of peptic ulcers, unless there's a very good reason, and person is also given misoprostol or PPI (H2 blockers don't reliably prevent NSAID ulcers) • Use one of the less toxic NSAIDS. (Best not to give NSAIDs at all to these pts) NSAIDs • NSAIDS cause mainly gastric ulcers, but can also cause duodenal ulcers and intestinal ulcers. NSAID ulcers are not associated with H. pylori. • Never give NSAIDS on the boards with warfarin. • Watch for NSAID induced interstitial nephritis (clue: electrolyte abnormalities, rising BUN or creat and urine eosinophils), nephrotic syndrome, hepatitis. NSAIDs • Most NSAIDs can cause heart failure (Celecoxib is possible exception). • Some NSAIDs are associated with MI (high dose ibuprofen, diclofenac, high dose celecoxib (200 mg bid), rofecoxib (off the market), but NOT naproxen, ?others (not enough data). • Most NSAIDs can cause hypertension (happens in first week) and mild transaminases elevation (10-15%). – Serious hepatitis is rare. • NSAIDs may cause non-unions and failure of bone grafts! Methotrexate • Common side effects are nausea, diarrhea, stomatitis, mild transaminase elevation, mild bone marrow suppression. • Folate supplementation prevents some of these. • 3%: pneumonitis and rarely pulmonary fibrosis. – Dry cough, fever, dyspnea, crackles Methotrexate • • • • Absolutely contraindicated in pregnancy. Avoid in drinkers and in those with chronic liver disease. Don’t use if creatinine > 2.0 mg/dl. Increasing MCV in a patient on methotrexate may be harbinger of more serious toxicity. Prednisone • Lupus patient receiving prednisone who develops "hip" pain (pain in groin and anteromedial thigh) • Get plain films followed by MRI to look for avascular necrosis. • Get MRI both sides (often asymptomatic on other side) Prednisone • Post-menopausal women receiving pred are at especially high risk or getting compression fractures. • Always get baseline DEXA. • Start alendronate or risedronate if osteopenic (T-score < -1). Prednisone • Steroid myopathy: patient on steroids is getting weaker (no pain). • CK is normal in steroid myopathy. • If appropriate do muscle biopsy (will show type II atrophy alone which is non-specific, but helpful to rule out other causes of myopathy). Prednisone • Serious long-term side effects include osteoporosis (with fractures, especially spinal compression fractures), cataracts (posterior sub-capsular if asked), glaucoma, poor healing, infections, avascular necrosis (up to 15% of lupus patients). Compression fractures can occur in 3 months in post-meno. women on pred. • A 30 year old woman with lupus limps into your office with right groin pain of 2 weeks duration. Meds include prednisone 10 mg/d and mycophenolate mofetil 1000 mg BID for lupus nephritis for the past six months. She is afebrile. Physical exam reveals severe pain with hip rotation. Right groin is tender to palpation overlying the hip joint. You order a radiograph which is read as normal. The most likely diagnosis is: A. B. C. D. Iliopsoas bursitis Avascular necrosis Septic arthritis Trochanteric bursitis • A 30 year old woman with lupus limps into your office with right groin pain of 2 weeks duration. Meds include prednisone 10 mg/d and mycophenolate mofetil 1000 mg BID for lupus nephritis for the past six months. She is afebrile. Physical exam reveals severe pain with hip rotation. Right groin is tender to palpation overlying the hip joint. You order a radiograph which is read as normal. The most likely diagnosis is: A. B. C. D. Iliopsoas bursitis Avascular necrosis Septic arthritis Trochanteric bursitis The “crescent sign” of AVN Bilateral hip AVN Prednisone • Watch on the boards for signs of acute adrenal insufficiency (ie., patient comes to ER having stopped prednisone (forgot to tell doctor) including nausea/vomiting, abdominal pain, hypotension, hypoglycemia, hyperkalemia, hi BUN, eosinophilia. Give saline and hydrocortisone. Chloroquine and Hydroxychloroquine • Useful for arthritis, skin. • Effective for prevention of renal relapses. • Common side-effects are blurry vision lasting two weeks at beginning of therapy (not serious), and generalized rash (stop drug). • Important: Serious but rare side-effect is maculopathy causing loss of central vision. Patient will notice that parts of words are missing when he/she reads. • Screen for maculopathy with ophthalmologic exam with red visual field testing once or twice yearly. Note that peripheral vision is not affected, thus this disease is not like glaucoma or pituitary tumor. Leflunomide A pyrimidine synthesis inhibitor. Indicated for RA. Very long half-life. Contraindicated in pregnancy. Not a good idea in liver disease or drinkers. • Side-effects very similar to methotrexate (nausea, diarrhea, alopecia, liver enzyme elevations, occasionally leukopenia. • • • • • The Biologics • Infliximab, adalimumab, golimumab, certolizumab, etanercept, anakinra, rituximab, tocilizumab and abatacept. • They are all expensive protein drugs. • They should not be combined with each other because of resulting serious immunosuppression! TNF Inhibitors Infliximab, Adalimumab, Etanercept, Certolizumab pegol, Golimumab • Used for rheumatoid arthritis, psoriasis, psoriatic arthritis, JRA, Crohn's disease and ulcerative colitis. • They retard joint damage and erosions. • Increased risk of bacterial and fungal infection. Discontinue during infections. • Associated with reactivation of TB, reactivation of hep B (but not hep C) and with malignancy. Do PPD first. TNF Inhibitors • Associated with development or reactivation of demyelinating disease such as multiple sclerosis. • Can cause heart failure (myocardial depressant). • May reactivate hepatitis B (not C). • Associated with development of anticardiolipin antibodies, ANA, anti-DNA and lupus-like syndrome, acute autoimmune hepatitis. Rituximab (Rituxan®) • B-cell killer. • Is an anti-CD-20 antibody. Depletes blood of B-cells for months. They eventually return. • Indicated for RA in combination with MTX, in patients having failed a TNF inhibitor. • Indicated for ANCA+ vasculitis. • Also indicated for CD20 positive B-cell lymphomas. • May cause hypogammaglobulinemia. Allopurinol • Most common side effect is rash. Don't restart it an allergic patient (could get Stevens-Johnson or toxic epidermal necrolysis). • Remember life-threatening interaction with azathioprine or 6-mercaptopurine. • "Allopurinol hypersensitivity syndrome" consists of fever, rash (often Stevens-Johnson), hepatitis, renal failure, leukocytosis, sometimes eosinophilia (the clue). • Reduce dose in renal insufficiency. • Never treat asymptomatic hyperuricemia on the boards (or in real life). Temporal Arteritis • Sed rate will almost always be very high on boards. • High sed rate, fever, weight loss and amaurosis fugax = temporal artery biopsy (even without headache). • Treat with prednisone 20 mg bid or tid to start. • Taper slowly, following ESR monthly. Temporal Arteritis • • • • • • Always do biopsy on the boards (and in real life) to confirm diagnosis. If offered, do bilateral biopsy. If only one side done and is negative, but patient really seems to have temporal arteritis, always biopsy other side. If neuro symptoms present (such as amaurosis fugax), begin treatment while waiting for biopsy, but not if only headache. A new-onset headache in an elderly person is always an indication for workup on the boards; it is never a tension headache. 30-40% present as polymyalgia rheumatica. A Case for the Boards • A 60 y.o. male gave a 3 year hx recurrent sinus congestion with crusting, conjunctival redness diagnosed as episcleritis. 1 month ago he developed acrocyanosis. 2 weeks ago he developed polyarthritis of all small joints. 1 week ago he developed fever and cough. CXR shows RUL infiltrate and mass. Bx showed granulomatous inflammation with negative AFB stain. Creatinine is 1.5 mg/dl, up from baseline of 0.9 mg/dl, and UA showed 40-50 RBCs with casts. Which of the following blood tests is most likely to be abnormal? A. B. C. D. E. P-ANCA C-ANCA ANA C3 Anti-MPO Which of the following blood tests is most likely to be abnormal? A. P-ANCA B. C-ANCA (and anti-PR3) C. ANA D. C3 E. Anti-MPO Kidney biopsy would likely show: A. B. C. D. Diffuse proliferative GN Membranous GN Paucimmune crescentic GN Membranoproliferative GN Kidney biopsy would likely show: A. B. C. D. Diffuse proliferative GN Membranous GN Paucimmune crescentic GN Membranoproliferative GN Kidney biopsy would likely show: A. B. C. D. Diffuse proliferative GN Membranous GN Paucimmune crescentic GN Membranoproliferative GN Definitive initial treatment would include: A. Daily prednisone alone B. Daily cyclosphosphamide alone C. Both A & B D. Pulse methylprednisolone alone Definitive initial treatment would include: A. Daily prednisone alone B. Daily cyclosphosphamide alone C. Both A & B D. Pulse methylprednisolone alone Wegener’s • Usually presents with sinus or URI symptoms. • Do c-ANCA (correlates with anti-proteinase 3 antibody). • Make every effort to confirm diagnosis with tissue before treating since treatment is not benign and C-ANCA not always reliable. • Biopsy sinus or lung usually. • Kidney biopsy not helpful (just shows glomerulonephritis). Wegener’s • Wegener's is the only vasculitis for which the initial treatment is cyclophosphamide. (Start prednisone with cyclophosphamide). • Often can switch to methotrexate after remission achieved. • Interstitial pneumonia developing in Wegener's patient on treatment is often P. carinii. • Other pulmonary-renal syndromes to consider include Goodpasture's (usually young men; antiGBM present), lupus, polyarteritis nodosa. Polyarteritis Nodosa • Key features will be mononeuritis multiplex (multiple individual peripheral nerve lesions [such as wrist drop, foot drop, patchy sensory loss, etc.) with weight loss, fever, high sed rate, abdominal pain, hypertension, hematuria, leukocytosis. • May have history IV amphetamine abuse or hepatitis B. Usually males. • Always biopsy (muscle, sural nerve, testicle) or do A-gram (mesenteric or renal) to confirm diagnosis. Don't biopsy kidney. • A. B. C. D. A 60-year-old male gives a one year history of recurrent sinusitis with persistent rhinorrhea and nasal crusting. He has had two episodes of otitis media in the past year (none previously) and one episode of chondritis of the helix that responded to prednisone. Recent urinalysis showed microscopic hematuria and 1+ proteinuria. Serum creatinine is 1.40 mg/dl (was 0.8 mg/dl last year). Which of the following antibodies is most likely to be elevated? Anti-DNA Anti-myeloperoxidase Anti-Smith Anti-proteinase-3 • A. B. C. D. A 60-year-old male gives a one year history of recurrent sinusitis with persistent rhinorrhea and nasal crusting. He has had two episodes of otitis media in the past year (none previously) and one episode of chondritis of the helix that responded to prednisone. Recent urinalysis showed microscopic hematuria and 1+ proteinuria. Serum creatinine is 1.40 mg/dl (was 0.8 mg/dl last year). Which of the following antibodies is most likely to be elevated? Anti-DNA Anti-myeloperoxidase (and P-ANCA) Anti-Smith Anti-proteinase-3 • Diseases often associated with positive ANCA include all of the following EXCEPT: – Wegener’s – Glomerulonephritis (some types) – Churg-Strauss vasculitis – Takayasu vasculitis – Microscopic polyarteritis Behcet’s Henoch-Schonlein • Key features will be arthralgias/arthritis, abdominal pain, heme positive stools, hematuria and a palpable purpura rash mainly on lower extremities and buttocks in a young adult. • (Most cases actually occur in children, but not on Internal Medicine boards). Initial treatment is supportive in children, but adults often require steroids. Takayasu’s • Key features will be upper extremity claudication, paresthesias, perhaps TIAs in a young woman with weight loss, fevers, high sed rate. • (These systemic features are often not present in real life, but probably will be on the boards). • Do aortic A-gram or MRA to confirm diagnosis. • Initial treatment is with steroids. Takayasu’s (left subclavian is missing) Hypersensitivity Vasculitis • Key features will be palpable purpura following drug administration (usually ß-lactam, phenytoin, sulfa, TNF inhibitors, etc.). • Do appropriate tests to rule out endocarditis, meningococcemia, RMSF (fever, myalgias, headache, rash in the summer in the ER on the boards is Rocky Mountain Spotted Fever until proved otherwise). • Treatment for hypersensitivity vasculitis due to drug is supportive (stop the drug!), not steroids, unless there is a clear indication. Primary CNS Vasculitis • Tip-off will be young person with stroke, high sed rate (not always present) and pleocytosis on LP. Note that diagnosis is by A-gram, or preferentially by leptomeningeal biopsy. Never Forgotten • Vasculitic rash on palms and soles? Always get RPR to rule out secondary syphilis. Multiple Cholesterol Emboli • Rare. Fever, weight loss, high sed rate, petechial rash on lower extremities, eosinophilia, and often some blue toes. Biopsy of the skin rash looking for cholesterol clefts in the small vessels is key to diagnosis. No effective treatment. Usually fatal. Self-perpetuating. • A "minor form" of this is the post-cath "blue toe syndrome," which usually resolves on its own. Ischemic Digits? Look For: • Vasculitis • Embolization (septic, non-septic) • Hypercoaguable state (including anti-cardiolipin antibodies) • Warfarin necrosis • Thromboangiitis obliterans • Scleroderma • Vasospasm. • In a word, blue digits can be vasculitic, vasospastic, clotted or obliterated. Single best test is an A-gram, but do as many blood tests as necessary. Pneumococcal sepsis (vasculitis mimic) Shoulder Pain • Most common cause of shoulder pain is not degenerative arthritis, but rather rotator cuff tendinitis, often with secondary subacromial bursitis. • Rotator cuff tendinitis often accompanied by impingement syndrome. Adduction of the raised arm often causes pain (impingement test), as does internal rotation while in abduction. • Pain radiating into the deltoid area is usually coming from the rotator cuff and/or subacromial bursa. Classic finding (ie., on the boards) is a "painful arc" between 60° - 120° of abduction. Shoulder Pain • Drop-arm test checks for large rotator cuff tear, but misses small or partial tears. • MRI is modality of choice to confirm tear; arthrogram or ultrasound also acceptable. • Not every rotator cuff tear needs operation, especially if patient is older. Conservative Rx first. Shoulder Pain • Oval, ill-defined, amorphous calcifications in region of humeral head are often seen in acute calcific tendinitis. May resolve spontaneously. • OK to inject these. Shoulder Pain • Bicipital tendinitis: tenderness of long head of biceps; positive Yergason test (Pain on resisted supination of forearm). • Bicipital tendon rupture may be only mildly painful; sudden appearance of palpable mass in upper arm (“Popeye sign”) Knee Pain • Patellofemoral pain syndrome: Bilateral knee pain (especially while descending steps) in young woman who may have some "knock-knee" deformity. May find patellofemoral tenderness and crepitus on exam, occasionally non-inflammatory effusions. Tx with PT, analgesic. Knee Pain • Torn meniscus. – Very common in elderly, usually nontraumatic. Sudden severe pain, especially on twisting. – Clue will be failure of DJD pain to improve with corticosteroid injection. – McMurray test – Diagnose by MRI. Knee Pain • Anserine bursitis. – Very common in middle-aged/elderly women with osteoarthritis of knee. – Key finding will be tenderness over medial tibia, about 2" below knee. – Corticosteroid injection helpful. • Prepatellar bursitis. – Often confused with joint effusion/arthritis. – Egg-shaped swelling superficial to patella. Usually post-traumatic ("housemaid's knee") – May also be septic or gouty. – Always tap and culture (on the boards), and only inject if certain no infection is present. Knee Pain • Baker's cyst - very popular on boards. – Pain in popliteal fossa, sometimes with swelling and paresthesias distally. – Clue is presence of knee effusion. Occurs in rheumatoid arthritis most commonly, but also in DJD. – If cyst ruptures, it mimics acute DVT with acute pain, redness, swelling (the fluid is very irritating to tissues). – The clue to rupture is presence of ecchymoses at ankle from blood/synovial fluid that flows along tissue plains following rupture. – Diagnose with MRI (best) or ultrasound (cheaper). Thoracic Back Pain • More ominous than low back pain! • Think: – Compression fracture – Malignancy – Dissecting aneurysm – MI • A 45 year old male presents on Monday morning with acute low back pain after digging six holes to plant trees in his yard two days prior. Mild radiation down right lower extremity to heel. No weakness. No bowel/bladder symptoms. On exam afebrile, reflexes intact. Mild spasm. Positive right-sided straight leg raising test. Which of the following is least appropriate at this time? A. 2 days of bedrest B. NSAID for pain relief C. Muscle relaxant for pain relief D. Lumbar AP & lateral radiographs E. Heating pad for pain relief • A 45 year old male presents on Monday morning with acute low back pain after digging six holes to plant trees in his yard two days prior. Mild radiation down right lower extremity to heel. No weakness. No bowel/bladder symptoms. On exam afebrile, reflexes intact. Mild spasm. Positive right-sided straight leg raising test. Which of the following is least appropriate at this time? A. 2 days of bedrest B. NSAID for pain relief C. Muscle relaxant for pain relief D. Lumbar AP & lateral radiographs E. Heating pad for pain relief Low Back Pain • The important causes of LBP for the boards are: – lumbosacral strain – spinal stenosis – herniated disc – Sacroiliitis – Malignancy – aortic aneurysm eroding vertebrae Low Back Pain • Initial treatment of uncomplicated back pain is two days of bedrest, antiinflammatories, pain meds, etc. Don't order x-rays for first episode of uncomplicated low back pain in relatively young, healthy persons. • In uncomplicated LBP, treatment precedes diagnosis (“lumbosacral strain”)! Low Back Pain • High risk persons with back pain: alcoholic, age over 50, trauma, corticosteroid use, high sed rate, fever. • Order x-rays for these pts. • If sed rate is very elevated in patient with back pain, always look for osteomyelitis, infectious discitis or malignancy. Low Back Pain • Spinal stenosis is common cause of LBP in older adults. • Presents with neurogenic claudication, a sensation of pain/weakness/rubbery feeling in buttocks and thighs on walking (mimics vascular claudication) relieved by sitting down or by bending forward. • Key differentiating point is patient can only stand 5-15 minutes in one place (unlike vascular claudication). • Diagnosis is by MRI. Tx is physical therapy, epidurals, surgery Low Back Pain • A positive straight leg raising test is pain going down leg (preferably to ankle) when the hip and the knee are extended. • Indicates nerve root irritation (most often seen with herniated disc) • Cauda equina syndrome is triad of impotence, sphincter dysfunction and "saddle anesthesia" occurring in setting of LBP. • Is usually neurosurgical emergency. Most common cause is large central disc herniation. Osteoarthritis • Risk Factors – – – – – – – – – – – – Age Female versus male sex Obesity Family history Lack of osteoporosis Occupation Sports activities Previous injury Muscle weakness Calcium crystal deposition disease Proprioceptive deficits Acromegaly DJD • Most specific finding on physical exam is crepitus. • Knee effusion common, and will be non-inflammatory (WBC < 250/mm3). • Common trouble spots are base of thumb (1st CMC joint), neck, lumbar, knees. DJD • Treatment includes (in order of use): – Acetaminophen – Quadriceps strengthening exercises (such as isometrics, swimming or bicycling) – Glucosamine (analgesic effect only), – NSAIDS – Corticosteroid injections – Viscosupplementation (intra-articular hyaluronic acid) – Cane – Total joint arthroplasty. DJD • Viscosupplementation: best for younger patients with mild-to-moderate cartilage loss. Pain improvement up to a year. • Has not been shown to decrease rate of joint space narrowing. • Given as a series of 3-5 intraarticular injections one week apart. May be repeated every 6-12 months. Diabetes – Musculoskeletal • Diabetics get frozen shoulder (also called adhesive capsulitis or periarthritis) (often bilateral). • Carpal tunnel syndrome • Ulnar neuropathy at elbow • Stiff, painful fingers often associated with sclerodactyly (diabetic cheiroarthropathy) • Dupuytren's contracture • Palmar tenosynovitis Diabetes - More • Charcot joint (mainly ankle and forefoot) • DISH (diffuse, idiopathic, skeletal hyperostosis [characterized by "candledripping syndesmophytes"]). • Back pain with anterior thigh atrophy in a diabetic is usually diabetic amyotrophy (ischemic lumbosacral plexopathy); usually resolves spontaneously, though not completely (don't be fooled and operate on "bulging discs" in such cases). Diabetes • Ischemic myopathy (thigh, forearm) – Normal A-gram usually – Very high CK – Poorly controlled diabetes Elbow pain • Lateral epicondylitis: Pain in lateral forearm while carrying objects with the forearm partially pronated (such as frying pans or milk bottles) • Confirm by finding tenderness in region of (and just distal to) the condyle. • Sometimes there is an associated radial nerve compression in the forearm also, but probably not on the boards. Elbow pain • An egg-shaped collection of fluid on the elbow is usually olecranon bursitis. It is usually painless. Always tap (if offered) to rule out infection, gout, rheumatoid arthritis, but most cases are posttraumatic or simply irritative in etiology. • Aspirate, use elbow pads; beware of injecting the infection rate is higher for this bursa. • Pain in 4th, 5th fingers raises question of ulnar neuropathy. Do Tinel's sign at the elbow to confirm diagnosis. Elbow pain • Radial nerve palsy will present with wrist drop on boards. • Patient may have fallen asleep on his/her arm (especially if intoxicated; “Saturday night palsy”) • Or could have polyarteritis nodosa. Carpal Tunnel Synd. • Carpal tunnel syndrome: Pain/tingling/numbness will be in first 3 fingers, not the wrist. • Patients like to "shake out" the hands in the morning, sometimes run under hot H2O. • Tinel sign: paresthesias in fingertips when the carpal tunnel is percussed with finger. • Phalen's test: Paresthesias when wrist is held in flexion for 30-60 seconds. If either positive, do EMG/NCS to confirm. Carpal Tunnel Synd. • Treatment is splinting first, not surgery. • Corticosteroid injection sometimes useful as diagnostic test. Always look for diabetes, hypothyroidism. In dialysis patients, may have ß2 microglobulinemia. Wrist Pain • Ganglion: firm yet movable nodule (few mm to 1 cm) on either side of wrist fixed to a tendon sheath (note the tendon sheath does not move with joint movement, only the tendon). • Treat conservatively or "needle" it. • Often regress or rupture spontaneously. Don't excise unless necessary. Causes of Hand Pain • • • • • • Carpal tunnel DJD RA Flexor tenosynovitis Hypertrophic pulm. osteoarthropathy Complex regional pain syndrome Hip Pain • Trochanteric bursitis. – Very common. – Often mistaken for DJD of hip joint. – Major symptom is inability to sleep on that side. – Pain is on lateral aspect of hip. Trochanter will be tender. – Treatment is corticosteroid injection just superficial to trochanter (and/or NSAID), rest. Hip Pain • Meralgia paresthetica: – Burning pain/numbness/paresthesias on anterolateral thigh due to compression of lateral femoral cutaneous nerve. Usually seen in obese, pregnant or diabetic patients, or due to tight belt or corset. Local measures and reassurance helpful. • Piriformis syndrome: – sciatica and buttock pain due to compression of sciatic nerve by piriformis muscle in mid-buttock. Look for in young women with abnormal gait who have sciatica with normal MRI of spine. Straight leg raising test usually negative. Plantar Fasciitis • Treatment of plantar fasciitis may include all of the following except: • Rest, ice, elevation • Stretching exercises • Night extension splint • Monthly corticosteroid injections • Silicone gel shoe inserts • Avoidance of barefoot walking • • • • • • • Your 65 y.o. patient with rheumatoid arthritis reports burning pain and numbness in the sole and toes of right foot after standing for 10 minutes, and at night. Inspection reveals mild swelling of the right ankle and the MTPs. Symptoms are reproducible by tapping with your finger posterior to the medial malleolus. You suspect one of the following: Morton’s neuroma Tarsal tunnel syndrome Plantar fasciitis Peripheral neuropathy Subtalar synovitis Carpal tunnel syndrome ACR classification criteria: Fibromyalgia • Both criteria must be satisfied – History of widespread pain for more than 3 months, on both sides of the body, above and below the waist, and axial skeleton (cervical spine, anterior chest, thoracic pain, or low back) – Pain in 11 of 18 tender point sites on digital palpation with approximate force of 4 kg. • Presence of second clinical disorder does not exclude diagnosis of fibromyalgia. NON-MUSCULOSKELETAL SYMPTOMS • • • • • • • Fatigue Sleep Disturbance Headaches GI symptoms (IBS) Irritable bladder Panic attacks (20) Vasospasm (40%) Dysmenorrhea Sicca symptoms Subjective swelling Numbness Affected by weather • AM stiffness • • • • • Fibromyalgia Treatment • Patient education; sleep hygiene • Pregabalin • Duloxetine • Tricyclics • Cyclobenzaprine • Note: SSRIs don’t work unless patient is depressed. Don’t Miss This Diagnosis • 70 year old female had left carpal tunnel surgery. One week later the entire hand swelled, became diffusely painful, became warm, seaty. The nails grew longer. Bone scan showed diffuse uptake (see scan). Hand became diffusely tender to palpation. The most likely diagnosis is: A. Carpal tunnel syndrome B. Rheumatoid arthritis C. Complex regional pain syndrome, type 1. D. Complex reginal pain syndrome, type 2. E. Pseudogout CRPS • Pain, burning • Vasomotor changes – – – – Temperature Color Piloerection Sweating • Often swelling • Type 1 – Trauma related • Type 1 – Non-trauma related CRPS • • • • • Early treatment is important Physical therapy Bisphosphonate Short course of steroids Anti-neuropathic (gabapentin, phenoxybenzamine) The End The End