Rheumatology Board Rheumatology Board ReviewReview

AM Report 6/19/09AM Report 6/19/09

http://photo.net/photodb/photo?photo_id=4509206&size=md

Arthritis affecting DIP JointArthritis affecting DIP Joint

Osteoarthritis (Heberden’s nodes)Osteoarthritis (Heberden’s nodes) Psoriatic arthritisPsoriatic arthritis

Arthritis affecting PIPsArthritis affecting PIPs

OA (Bouchard’s nodes)OA (Bouchard’s nodes) RARA SLESLE SclerodermaScleroderma

Arthritis affecting MCPsArthritis affecting MCPs

RARA SLESLE SclerodermaScleroderma HemachromatosisHemachromatosis

Causes of Monoarticular Causes of Monoarticular ArthritisArthritis

TraumaTrauma InfectionInfection CrystalsCrystals

AKA 3 Bs: blood, bugs, birefringenceAKA 3 Bs: blood, bugs, birefringence Key to diagnosis: arthrocentesis!!Key to diagnosis: arthrocentesis!!

64 M with chronic gout presents with swollen right 64 M with chronic gout presents with swollen right elbow of two days duration. No trauma. Last gout elbow of two days duration. No trauma. Last gout attack involved right knee 4 months ago. attack involved right knee 4 months ago. On exam, Temp 38.1. No LAD. Right elbow warm On exam, Temp 38.1. No LAD. Right elbow warm with minimal erythema. MSK exam reveals fullness with minimal erythema. MSK exam reveals fullness and tenderness over right olecranon process. and tenderness over right olecranon process. Which is most important next step in patient’s Which is most important next step in patient’s management?management?

A> Right elbow joint aspirationA> Right elbow joint aspiration B> Right olecrenon bursa aspirationB> Right olecrenon bursa aspiration C> Radiography of right elbow and C> Radiography of right elbow and

forearmforearm D> Erythrocyte sedimentation rateD> Erythrocyte sedimentation rate

B> Immediate aspiration of olecranon B> Immediate aspiration of olecranon bursabursa

Olecranon bursitis may be infectious, Olecranon bursitis may be infectious, crystalline, or traumaticcrystalline, or traumatic

Bursa aspiration is indicated for Bursa aspiration is indicated for patients with acute olecranon bursitis patients with acute olecranon bursitis to guide therapyto guide therapy

Trauma is the most common cause Trauma is the most common cause of olecranon bursitis, but other of olecranon bursitis, but other causes must be ruled out. causes must be ruled out.

45 F has 6-month history joint pain and fatigue. 45 F has 6-month history joint pain and fatigue. First she developed pain, then swelling and redness First she developed pain, then swelling and redness of fifth right hand DIP, 4of fifth right hand DIP, 4thth DIP left hand, right wrist, DIP left hand, right wrist, third and fourth left toes. Which is most likely third and fourth left toes. Which is most likely

diagnosis?diagnosis?

A> Rheumatoid arthritisA> Rheumatoid arthritis B> SarcoidosisB> Sarcoidosis C> Wegener’s granulomatosisC> Wegener’s granulomatosis D> Psoriatic arthritisD> Psoriatic arthritis

D> Psoriatic ArthritisD> Psoriatic Arthritis

Photo demonstrates dactylitis (sausage digit), Photo demonstrates dactylitis (sausage digit), which is common in psoriatic arthritis (also which is common in psoriatic arthritis (also sometimes present in sarcoidosis)sometimes present in sarcoidosis)

PA is classically found in distal joints, in PA is classically found in distal joints, in asymmetric patternasymmetric pattern

15% develop joint involvement before skin 15% develop joint involvement before skin involvementinvolvement

20% are HLA-B27 positive20% are HLA-B27 positive

Bonus: What is treatment for PA?Bonus: What is treatment for PA?

Treatment of psoriatic arthritisTreatment of psoriatic arthritis

Like RA, disease is T-cell mediated, Like RA, disease is T-cell mediated, so therapy is similar to RAso therapy is similar to RA

NSAIDS and low-dose prednisoneNSAIDS and low-dose prednisone MethotrexateMethotrexate TNF-alpha antagonistsTNF-alpha antagonists

67 M has three-month history of painful paresthesias on 67 M has three-month history of painful paresthesias on dorsum of right foot. He now stumbles because he is unable to dorsum of right foot. He now stumbles because he is unable to fully raise foot. Also complains of fevers, night sweats, fully raise foot. Also complains of fevers, night sweats, arthralgias, myalgias. arthralgias, myalgias. On physical exam, he has livedo reticularis on LEs. On MSK On physical exam, he has livedo reticularis on LEs. On MSK exam, unable to dorsiflex right foot. Labs: exam, unable to dorsiflex right foot. Labs: Hgb 10.5 g/dLHgb 10.5 g/dLESR 98 mm/hESR 98 mm/hANA negativeANA negativeANCA negativeANCA negativeHBV Sag positiveHBV Sag positiveHBV S Ab negativeHBV S Ab negativeHBV core Ab positiveHBV core Ab positiveHCV Ab negativeHCV Ab negativeCryoglobulins negativeCryoglobulins negative

Diagnosis?Diagnosis? A> polyarteritis nodosaA> polyarteritis nodosa B> Acute Hepatitis B infectionB> Acute Hepatitis B infection C> SclerodermaC> Scleroderma D> Wegener’s granulomatosisD> Wegener’s granulomatosis E> Cryoglobulinemic vasculitisE> Cryoglobulinemic vasculitis

Polyarteritis NodosaPolyarteritis Nodosa

Necrotizing vasculitis of small and medium-sized Necrotizing vasculitis of small and medium-sized arteriesarteries

Mononeuritis Multiplex-Mononeuritis Multiplex- common presenting common presenting symptom, present in 60%symptom, present in 60%

Associated with HBVAssociated with HBV Other clinical signs: HTN, fever, MSK symptoms, Other clinical signs: HTN, fever, MSK symptoms,

involvement of GI, nervous system, heart, involvement of GI, nervous system, heart, nonglomerular renal vesselsnonglomerular renal vessels

Other: how do you make diagnosis?Other: how do you make diagnosis? How to treat? How to treat?

PANPAN

Diagnosis: biopsy or arteriography if Diagnosis: biopsy or arteriography if biopsy not possible. Arteriography biopsy not possible. Arteriography usually done of visceral and renal usually done of visceral and renal vesselsvessels

Treatment: cyclophosphamide, Treatment: cyclophosphamide, prednisone, and antiviral tx of HBV or prednisone, and antiviral tx of HBV or HIVHIV

51 M with 4-year history of diffuse cutaneous sytemic 51 M with 4-year history of diffuse cutaneous sytemic sclerosois is hospitalized for new-onset HTN associated with sclerosois is hospitalized for new-onset HTN associated with anemia and thrombocytopenia. At time of admission, captopril anemia and thrombocytopenia. At time of admission, captopril 6.25 mg q8 hrs was initiated, which was titrated up to 25 mg 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 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?1.4 to 2.1 mg/dL. What is the most appropriate management?

A> Discontinue captopril, start calcium channel A> Discontinue captopril, start calcium channel blockerblocker

B> Continue to increase captopril doseB> Continue to increase captopril dose C> perform plasmapheresisC> perform plasmapheresis D> perform captopril renographyD> perform captopril renography

B> continue to increase captopril doseB> continue to increase captopril dose

Pt has Pt has Scleroderma Renal CrisisScleroderma Renal Crisis SRC is characterized by HTN, hemolytic SRC is characterized by HTN, hemolytic

anemia, thrombocytopenia, non-oliguric anemia, thrombocytopenia, non-oliguric renal failurerenal failure

ACE Inhibitors are the most effective for ACE Inhibitors are the most effective for improving renal function in SRCimproving renal function in SRC

ACE-I should be continued even when ACE-I should be continued even when creatinine levels continue to rise and creatinine levels continue to rise and patient requires hemodialysispatient requires hemodialysis

SclerodermaScleroderma

Most frequent in ages 30-Most frequent in ages 30-50, 3:1 female 50, 3:1 female

Diffuse vs limited (CREST)Diffuse vs limited (CREST) In CREST, skin thickening In CREST, skin thickening

found only distal to elbows found only distal to elbows and kneesand knees

Diagnosis is clinical, Diagnosis is clinical, supported by positive ANA, supported by positive ANA, Anti-Scl-70 abs, anti-Anti-Scl-70 abs, anti-centromere abscentromere abs

Anti-inflammatory Anti-inflammatory treatment not useful. Treat treatment not useful. Treat individual complicationsindividual complications

Scleroderma Pulmonary Scleroderma Pulmonary DiseaseDisease

Primary cause of mortalityPrimary cause of mortality Interstitial lung disease and Interstitial lung disease and

pulmonary artery HTN (PAH)pulmonary artery HTN (PAH)

Tx for ILD: cyclophosphamideTx for ILD: cyclophosphamide Tx for PAH: warfarin, home O2, Tx for PAH: warfarin, home O2,

endothelin-receptor antagonist endothelin-receptor antagonist (bosentan) or prostacyclin analog(bosentan) or prostacyclin analog

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

A> sacral fractureA> sacral fracture B> ankylosing B> ankylosing

spondylitisspondylitis C> osteoarthritisC> osteoarthritis D> metastatic D> metastatic

cancercancer

Ankylosing SpondylitisAnkylosing Spondylitis

9:1 male predominance9:1 male predominance Systemic: fatigue, weight loss, Systemic: fatigue, weight loss,

anemia, elevated ESR and CRPanemia, elevated ESR and CRP Primarily affects spine and SI jointsPrimarily affects spine and SI joints Extraspinal: uveitis (40%), Extraspinal: uveitis (40%),

enthesitis/heel pain, hip or shoulder enthesitis/heel pain, hip or shoulder painpain

Treatment: NSAIDS, anti-TNFTreatment: NSAIDS, anti-TNF

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

39 F presents with 2-month history of PIP and wrist 39 F presents with 2-month history of PIP and wrist swelling, pain. Some AM stiffness. swelling, pain. Some AM stiffness. PE: mild symmetric synovitis of MCPs, wrists, kneesPE: mild symmetric synovitis of MCPs, wrists, kneesLabs:Labs:Hgb: 10.9 g/dLHgb: 10.9 g/dLRheumatoid Factor: negativeRheumatoid Factor: negativeANA : negativeANA : negativeAnti-CCP: PositiveAnti-CCP: PositiveWhat is most likely diagnosis?What is most likely diagnosis?

A>OsteoarthritisA>Osteoarthritis B>Psoriatic arthritisB>Psoriatic arthritis C>Rheumatoid arthritisC>Rheumatoid arthritis D>Calcium Pyrophosphate Deposition DzD>Calcium Pyrophosphate Deposition Dz

C> Rheumatoid ArthritisC> Rheumatoid Arthritis

RF is present in 85% of patients with RF is present in 85% of patients with RA, but only 33% are positive within RA, but only 33% are positive within first six months months of diseasefirst six months months of disease

Anti-CCP is 95% specific for RAAnti-CCP is 95% specific for RA

Diagnosis of RA: Need 4 of 7Diagnosis of RA: Need 4 of 7

Symptoms > 6 weeksSymptoms > 6 weeks Morning stiffness> 1 hourMorning stiffness> 1 hour 3 or more affected joints3 or more affected joints Joints include wrists, MCP, PIPJoints include wrists, MCP, PIP + rheumatoid nodules+ rheumatoid nodules +rheumatoid factor+rheumatoid factor Radiographic changesRadiographic changes

75 F presents with fever, malaise, severe HA, jaw 75 F presents with fever, malaise, severe HA, jaw pain when chewing. pain when chewing. PE: PE: T 100.1. +scalp tendernessT 100.1. +scalp tendernessLabs:Labs:ESR 30ESR 30Temporal artery biopsy will be performed in four Temporal artery biopsy will be performed in four days. days.

What is most appropriate management until biopsy What is most appropriate management until biopsy is performed?is performed?

A> Low molecular weight heparinA> Low molecular weight heparin B> PrednisoneB> Prednisone C> Methotrexate and prednisoneC> Methotrexate and prednisone D> AcetaminophenD> Acetaminophen E> No therapy until biopsy results availableE> No therapy until biopsy results available

B> prednisoneB> prednisone

Pt likely has GCA (temporal arteritis), a Pt likely has GCA (temporal arteritis), a vasculitis of branches or carotid arteryvasculitis of branches or carotid artery

Immediate prednisone therapy is Immediate prednisone therapy is indicated for any pts with strong clinical indicated for any pts with strong clinical suspicionsuspicion

Diagnosis is by temporal artery biopsyDiagnosis is by temporal artery biopsy Prednisone therapy up to two weeks Prednisone therapy up to two weeks

prior to biopsy will not affect biopsy prior to biopsy will not affect biopsy resultsresults

MTX has no role in treatment of GCAMTX has no role in treatment of GCA GCA has strong correlation with PMRGCA has strong correlation with PMR

48 M evaluated for annual physical. He has history of HTN and 48 M evaluated for annual physical. He has history of HTN and tophaceous gout. Last gout attack was mild and happened six tophaceous gout. Last gout attack was mild and happened six months ago. months ago. Meds: enalapril 20 qd, colchicine 0.6 mg qd, allopurinol 300 Meds: enalapril 20 qd, colchicine 0.6 mg qd, allopurinol 300 mg qdmg qdExam: non-tender olecranon nodules and slightly tender first Exam: non-tender olecranon nodules and slightly tender first MTPMTPLabs: urate 7.2 mg /dLLabs: urate 7.2 mg /dL

What is appropriate next step in management?What is appropriate next step in management? A> Discontinue colchicineA> Discontinue colchicine B> D/c colchicine, increase allopurinol to B> D/c colchicine, increase allopurinol to

400 mg/d400 mg/d C> Increase allopurinol to 400 mg/dC> Increase allopurinol to 400 mg/d D> Obtain 24 hr urine urate excretionD> Obtain 24 hr urine urate excretion

C> Increase allopurinol dose to 400 mg/dC> Increase allopurinol dose to 400 mg/d

Goal of hypouricemic therapy is to Goal of hypouricemic therapy is to reduce urate to 6 mg/dLreduce urate to 6 mg/dL

Urate will continue to deposit in Urate will continue to deposit in tissues when serum urate >6.8 mg/dLtissues when serum urate >6.8 mg/dL

A follow-up urate level should be A follow-up urate level should be checkedchecked

Cochicine should be continued Cochicine should be continued because increasing allopurinol may because increasing allopurinol may trigger a gout attacktrigger a gout attack

62 M presents with right knee pain and 62 M presents with right knee pain and swelling for three days. He has had swelling for three days. He has had recurrent episodes lasting several days for recurrent episodes lasting several days for past five years. past five years. Exam: moderate synovitis and joint effusion.Exam: moderate synovitis and joint effusion.

Diagnosis? Diagnosis?

A> CPPDA> CPPD B> OsteoarthritisB> Osteoarthritis C> Anserine bursitisC> Anserine bursitis D> GoutD> Gout E> Gonococcal arthritisE> Gonococcal arthritis

A> A> CPPDCPPD Chondrocalcinosis: calcification of Chondrocalcinosis: calcification of

cartilage, here in knee meniscus, cartilage, here in knee meniscus, pathognomic for CPPDpathognomic for CPPD

Diagnosis of CPPD: calcium pyrophosphate Diagnosis of CPPD: calcium pyrophosphate crystals on fluid analysis (weakly crystals on fluid analysis (weakly birefringent)birefringent)

CPPD may occur with normal serum CPPD may occur with normal serum calcium levelscalcium levels

Treatment: prednisone, colchicineTreatment: prednisone, colchicine Remember: acute monoarticular arthritis Remember: acute monoarticular arthritis

requires arthrocentesis!!requires arthrocentesis!!

32 F presents with 5 months of pain and swelling in hands, 32 F presents with 5 months of pain and swelling in hands, progressive weakness. Difficult to get in bathtub, hold progressive weakness. Difficult to get in bathtub, hold hairdryer, walk up stairs due to weakness. hairdryer, walk up stairs due to weakness. PE: puffy hands with scaly, darkened fingers. Bibasilar PE: puffy hands with scaly, darkened fingers. Bibasilar crackles. Proximal weakness upper and lower extremities.crackles. Proximal weakness upper and lower extremities.Labs:Labs:CBC normal. Creatinine normal.CBC normal. Creatinine normal.ESR 24ESR 24CK 4250 U/LCK 4250 U/LANA 1:160ANA 1:160

Diagnosis?Diagnosis? A> polymyalgia rheumaticaA> polymyalgia rheumatica B> SLEB> SLE C> MCTDC> MCTD D> PolymyositisD> Polymyositis E> SclerodermaE> Scleroderma

PolymyositisPolymyositis

Proximal muscle weakness without Proximal muscle weakness without pain (contrast to PMR)pain (contrast to PMR)

Diagnosis: elevated CK, ANA (80%), Diagnosis: elevated CK, ANA (80%), EMG, Muscle biopsyEMG, Muscle biopsy

Anti-Jo-1 Abs: found in variant of Anti-Jo-1 Abs: found in variant of polymyositis with ILD, mechanics’ polymyositis with ILD, mechanics’ hands, polyarthritis, Raynaud’shands, polyarthritis, Raynaud’s