1. Rheumatology Board Review AM Report 6/19/09

3. Arthritis affecting DIP Joint Osteoarthritis (Heberden’s nodes) Osteoarthritis (Heberden’s nodes) Psoriatic arthritis Psoriatic arthritis

4. Arthritis affecting PIPs OA (Bouchard’s nodes) OA (Bouchard’s nodes) RA RA SLE SLE Scleroderma Scleroderma

5. Arthritis affecting MCPs RA RA SLE SLE Scleroderma Scleroderma Hemachromatosis Hemachromatosis

6. Causes of Monoarticular Arthritis Trauma Trauma Infection Infection Crystals Crystals AKA 3 Bs: blood, bugs, birefringence AKA 3 Bs: blood, bugs, birefringence Key to diagnosis: arthrocentesis!! Key to diagnosis: arthrocentesis!!

7. 64 M with chronic gout presents with swollen right elbow of two days duration. No trauma. Last gout attack involved right knee 4 months ago. On exam, Temp 38.1. No LAD. Right elbow warm with minimal erythema. MSK exam reveals fullness and tenderness over right olecranon process. Which is most important next step in patient’s management? A> Right elbow joint aspiration A> Right elbow joint aspiration B> Right olecrenon bursa aspiration B> Right olecrenon bursa aspiration C> Radiography of right elbow and forearm C> Radiography of right elbow and forearm D> Erythrocyte sedimentation rate D> Erythrocyte sedimentation rate

8. B> Immediate aspiration of olecranon bursa Olecranon bursitis may be infectious, crystalline, or traumatic Olecranon bursitis may be infectious, crystalline, or traumatic Bursa aspiration is indicated for patients with acute olecranon bursitis to guide therapy Bursa aspiration is indicated for patients with acute olecranon bursitis to guide therapy Trauma is the most common cause of olecranon bursitis, but other causes must be ruled out. Trauma is the most common cause of olecranon bursitis, but other causes must be ruled out.

9. 45 F has 6-month history joint pain and fatigue. First she developed pain, then swelling and redness of fifth right hand DIP, 4 th DIP left hand, right wrist, third and fourth left toes. Which is most likely diagnosis? A> Rheumatoid arthritis A> Rheumatoid arthritis B> Sarcoidosis B> Sarcoidosis C> Wegener’s granulomatosis C> Wegener’s granulomatosis D> Psoriatic arthritis D> Psoriatic arthritis

10. D> Psoriatic Arthritis Photo demonstrates dactylitis (sausage digit), which is common in psoriatic arthritis (also sometimes present in sarcoidosis) Photo demonstrates dactylitis (sausage digit), which is common in psoriatic arthritis (also sometimes present in sarcoidosis) PA is classically found in distal joints, in asymmetric pattern PA is classically found in distal joints, in asymmetric pattern 15% develop joint involvement before skin involvement 15% develop joint involvement before skin involvement 20% are HLA-B27 positive 20% are HLA-B27 positive Bonus: What is treatment for PA? Bonus: What is treatment for PA?

11. Treatment of psoriatic arthritis Like RA, disease is T-cell mediated, so therapy is similar to RA Like RA, disease is T-cell mediated, so therapy is similar to RA NSAIDS and low-dose prednisone NSAIDS and low-dose prednisone Methotrexate Methotrexate TNF-alpha antagonists TNF-alpha antagonists

12. 67 M has three-month history of painful paresthesias on dorsum of right foot. He now stumbles because he is unable to fully raise foot. Also complains of fevers, night sweats, arthralgias, myalgias. On physical exam, he has livedo reticularis on LEs. On MSK exam, unable to dorsiflex right foot. Labs: Hgb 10.5 g/dL ESR 98 mm/h ANA negative ANCA negative HBV Sag positive HBV S Ab negative HBV core Ab positive HCV Ab negative Cryoglobulins negative Diagnosis? A> polyarteritis nodosa A> polyarteritis nodosa B> Acute Hepatitis B infection B> Acute Hepatitis B infection C> Scleroderma C> Scleroderma D> Wegener’s granulomatosis D> Wegener’s granulomatosis E> Cryoglobulinemic vasculitis E> Cryoglobulinemic vasculitis

13. Polyarteritis Nodosa Necrotizing vasculitis of small and medium-sized arteries Necrotizing vasculitis of small and medium-sized arteries Mononeuritis Multiplex- common presenting symptom, present in 60% Mononeuritis Multiplex- common presenting symptom, present in 60% Associated with HBV Associated with HBV Other clinical signs: HTN, fever, MSK symptoms, involvement of GI, nervous system, heart, nonglomerular renal vessels Other clinical signs: HTN, fever, MSK symptoms, involvement of GI, nervous system, heart, nonglomerular renal vessels Other: how do you make diagnosis? Other: how do you make diagnosis? How to treat? How to treat?

14. PAN Diagnosis: biopsy or arteriography if biopsy not possible. Arteriography usually done of visceral and renal vessels Diagnosis: biopsy or arteriography if biopsy not possible. Arteriography usually done of visceral and renal vessels Treatment: cyclophosphamide, prednisone, and antiviral tx of HBV or HIV Treatment: cyclophosphamide, prednisone, and antiviral tx of HBV or HIV

15. 51 M with 4-year history of diffuse cutaneous sytemic sclerosois is hospitalized for new-onset HTN associated with anemia and thrombocytopenia. At time of admission, captopril 6.25 mg q8 hrs was initiated, which was titrated up to 25 mg q8. Three days later, BP down to 140/80 but Cr increased from 1.4 to 2.1 mg/dL. What is the most appropriate management? A> Discontinue captopril, start calcium channel blocker A> Discontinue captopril, start calcium channel blocker B> Continue to increase captopril dose B> Continue to increase captopril dose C> perform plasmapheresis C> perform plasmapheresis D> perform captopril renography D> perform captopril renography

16. B> continue to increase captopril dose Pt has Scleroderma Renal Crisis Pt has Scleroderma Renal Crisis SRC is characterized by HTN, hemolytic anemia, thrombocytopenia, non-oliguric renal failure SRC is characterized by HTN, hemolytic anemia, thrombocytopenia, non-oliguric renal failure ACE Inhibitors are the most effective for improving renal function in SRC ACE Inhibitors are the most effective for improving renal function in SRC ACE-I should be continued even when creatinine levels continue to rise and patient requires hemodialysis ACE-I should be continued even when creatinine levels continue to rise and patient requires hemodialysis

17. Scleroderma Most frequent in ages 30-50, 3:1 female Most frequent in ages 30-50, 3:1 female Diffuse vs limited (CREST) Diffuse vs limited (CREST) In CREST, skin thickening found only distal to elbows and knees In CREST, skin thickening found only distal to elbows and knees Diagnosis is clinical, supported by positive ANA, Anti-Scl-70 abs, anti-centromere abs Diagnosis is clinical, supported by positive ANA, Anti-Scl-70 abs, anti-centromere abs Anti-inflammatory treatment not useful. Treat individual complications Anti-inflammatory treatment not useful. Treat individual complications

18. Scleroderma Pulmonary Disease Primary cause of mortality Primary cause of mortality Interstitial lung disease and pulmonary artery HTN (PAH) Interstitial lung disease and pulmonary artery HTN (PAH) Tx for ILD: cyclophosphamide Tx for ILD: cyclophosphamide Tx for PAH: warfarin, home O2, endothelin-receptor antagonist (bosentan) or prostacyclin analog Tx for PAH: warfarin, home O2, endothelin-receptor antagonist (bosentan) or prostacyclin analog

19. 22 F evaluated for 12 month history of worsening low back stiffness which is present for two hours each AM. +fatigue. No fever, chills, weight loss, night sweats. One year ago, had uveitis of right eye. PE: mild forward bending of spine. L-spine and SI joints are tender. Decreased chest expansion. Labs: hgb 12.5 g/dL. ESR 85 mm/hr. Diagnosis? A> sacral fracture A> sacral fracture B> ankylosing spondylitis B> ankylosing spondylitis C> osteoarthritis C> osteoarthritis D> metastatic cancer D> metastatic cancer

20. Ankylosing Spondylitis 9:1 male predominance 9:1 male predominance Systemic: fatigue, weight loss, anemia, elevated ESR and CRP Systemic: fatigue, weight loss, anemia, elevated ESR and CRP Primarily affects spine and SI joints Primarily affects spine and SI joints Extraspinal: uveitis (40%), enthesitis/heel pain, hip or shoulder pain Extraspinal: uveitis (40%), enthesitis/heel pain, hip or shoulder pain Treatment: NSAIDS, anti-TNF Treatment: NSAIDS, anti-TNF

21. Seronegative Spondyloarthropathies: group of sytemic inflammatory joint disorders that share clinical, radiologicial, genetic features. Strong correlation with HLA-B27 antigen. Commonly have back pain with morning stiffness and sacroiliitis Ankylosing spondylitis Ankylosing spondylitis Reactive arthritis (Reiter’s) Reactive arthritis (Reiter’s) Psoriatic arthritis Psoriatic arthritis Enteropathic arthritis Enteropathic arthritis

22. 39 F presents with 2-month history of PIP and wrist swelling, pain. Some AM stiffness. PE: mild symmetric synovitis of MCPs, wrists, knees Labs: Hgb: 10.9 g/dL Rheumatoid Factor: negative ANA : negative Anti-CCP: Positive What is most likely diagnosis? A>Osteoarthritis A>Osteoarthritis B>Psoriatic arthritis B>Psoriatic arthritis C>Rheumatoid arthritis C>Rheumatoid arthritis D>Calcium Pyrophosphate Deposition Dz D>Calcium Pyrophosphate Deposition Dz

23. C> Rheumatoid Arthritis RF is present in 85% of patients with RA, but only 33% are positive within first six months months of disease RF is present in 85% of patients with RA, but only 33% are positive within first six months months of disease Anti-CCP is 95% specific for RA Anti-CCP is 95% specific for RA

24. Diagnosis of RA: Need 4 of 7 Symptoms > 6 weeks Symptoms > 6 weeks Morning stiffness> 1 hour Morning stiffness> 1 hour 3 or more affected joints 3 or more affected joints Joints include wrists, MCP, PIP Joints include wrists, MCP, PIP + rheumatoid nodules + rheumatoid nodules +rheumatoid factor +rheumatoid factor Radiographic changes Radiographic changes

25. 75 F presents with fever, malaise, severe HA, jaw pain when chewing. PE: T 100.1. +scalp tenderness Labs: ESR 30 Temporal artery biopsy will be performed in four days. What is most appropriate management until biopsy is performed? A> Low molecular weight heparin A> Low molecular weight heparin B> Prednisone B> Prednisone C> Methotrexate and prednisone C> Methotrexate and prednisone D> Acetaminophen D> Acetaminophen E> No therapy until biopsy results available E> No therapy until biopsy results available

26. B> prednisone Pt likely has GCA (temporal arteritis), a vasculitis of branches or carotid artery Pt likely has GCA (temporal arteritis), a vasculitis of branches or carotid artery Immediate prednisone therapy is indicated for any pts with strong clinical suspicion Immediate prednisone therapy is indicated for any pts with strong clinical suspicion Diagnosis is by temporal artery biopsy Diagnosis is by temporal artery biopsy Prednisone therapy up to two weeks prior to biopsy will not affect biopsy results Prednisone therapy up to two weeks prior to biopsy will not affect biopsy results MTX has no role in treatment of GCA MTX has no role in treatment of GCA GCA has strong correlation with PMR GCA has strong correlation with PMR

27. 48 M evaluated for annual physical. He has history of HTN and tophaceous gout. Last gout attack was mild and happened six months ago. Meds: enalapril 20 qd, colchicine 0.6 mg qd, allopurinol 300 mg qd Exam: non-tender olecranon nodules and slightly tender first MTP Labs: urate 7.2 mg /dL What is appropriate next step in management? A> Discontinue colchicine A> Discontinue colchicine B> D/c colchicine, increase allopurinol to 400 mg/d B> D/c colchicine, increase allopurinol to 400 mg/d C> Increase allopurinol to 400 mg/d C> Increase allopurinol to 400 mg/d D> Obtain 24 hr urine urate excretion D> Obtain 24 hr urine urate excretion

28. C> Increase allopurinol dose to 400 mg/d Goal of hypouricemic therapy is to reduce urate to 6 mg/dL Goal of hypouricemic therapy is to reduce urate to 6 mg/dL Urate will continue to deposit in tissues when serum urate >6.8 mg/dL Urate will continue to deposit in tissues when serum urate >6.8 mg/dL A follow-up urate level should be checked A follow-up urate level should be checked Cochicine should be continued because increasing allopurinol may trigger a gout attack Cochicine should be continued because increasing allopurinol may trigger a gout attack

29. 62 M presents with right knee pain and swelling for three days. He has had recurrent episodes lasting several days for past five years. Exam: moderate synovitis and joint effusion. Diagnosis? A> CPPD A> CPPD B> Osteoarthritis B> Osteoarthritis C> Anserine bursitis C> Anserine bursitis D> Gout D> Gout E> Gonococcal arthritis E> Gonococcal arthritis

30. A> CPPD Chondrocalcinosis: calcification of cartilage, here in knee meniscus, pathognomic for CPPD Chondrocalcinosis: calcification of cartilage, here in knee meniscus, pathognomic for CPPD Diagnosis of CPPD: calcium pyrophosphate crystals on fluid analysis (weakly birefringent) Diagnosis of CPPD: calcium pyrophosphate crystals on fluid analysis (weakly birefringent) CPPD may occur with normal serum calcium levels CPPD may occur with normal serum calcium levels Treatment: prednisone, colchicine Treatment: prednisone, colchicine Remember: acute monoarticular arthritis requires arthrocentesis!! Remember: acute monoarticular arthritis requires arthrocentesis!!

31. 32 F presents with 5 months of pain and swelling in hands, progressive weakness. Difficult to get in bathtub, hold hairdryer, walk up stairs due to weakness. PE: puffy hands with scaly, darkened fingers. Bibasilar crackles. Proximal weakness upper and lower extremities. Labs: CBC normal. Creatinine normal. ESR 24 CK 4250 U/L ANA 1:160 Diagnosis? A> polymyalgia rheumatica A> polymyalgia rheumatica B> SLE B> SLE C> MCTD C> MCTD D> Polymyositis D> Polymyositis E> Scleroderma E> Scleroderma

32. Polymyositis Proximal muscle weakness without pain (contrast to PMR) Proximal muscle weakness without pain (contrast to PMR) Diagnosis: elevated CK, ANA (80%), EMG, Muscle biopsy Diagnosis: elevated CK, ANA (80%), EMG, Muscle biopsy Anti-Jo-1 Abs: found in variant of polymyositis with ILD, mechanics’ hands, polyarthritis, Raynaud’s Anti-Jo-1 Abs: found in variant of polymyositis with ILD, mechanics’ hands, polyarthritis, Raynaud’s