abim logy review 2010

8/9/2019 ABIM logy Review 2010

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RHEUMATOLOGY SECTIONABIM

BOARD REVIEW

Matt Husa, M.D.

UCSD Rheumatology Fellow

4/09/10


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Board Overview

Know a few diseasesdiagnosis more

important than treatment

Diagnosis: history, physical exam, imaging,labs

Some pathophysiology

Some common treatments/sideeffects/contraindications

Serologies


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Overview: Topics to Study

RA RA vs. seronegative

Mgmt of RATNFinhibitors

OA

Crystal disease SLE

Pregnancy in SLE/autoimmune disease

Management of monoarticular arthritis

Vasculitis differential diagnosisANCA, pulm/renalsyndrome

Serologies

2-3qu on less common rheumatic disease


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Rheum Clinical Pearls

Arthritis vs. arthralgias

Morning stiffness resolves w/ movement, >30min is significant

Malar erythema spares nasolabial folds

Objective muscular weakness: inflammatory myositis vs.

myopathy (PMR is achey pain, not objective weakness)

Hip pain: groin area, internal/external rotation (lateral thigh pain =

trochanteric bursitis)

DIP arthritis: CPPD/Gout vs. psoriatic arthritis vs. osteoarthritis

Fever in rheumatic disease: SLE vs. vasculitis vs. adult onset

Stills disease vs. periodic familial fever syndromes vs. crystalarthropathy (less in others)

Uveitis and arthritis: seronegatives, Wegeners, Behcets, IBD

Arthritis and lung disease: scleroderma (ILD and PAH), SLE

(PAH), inflam myopathy (ILD), AS (upper lobes), vasculitis


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Labs in Rheumatology

ESR/CRP: non-specific markers of inflammation,valuable in RA, PMR, vasculitis, otherwise not

that helpful.

CBC: lymphopenia, leukopenia, anemia chronic

disease, platelets increased or decreased

RF and anti-cyclic citrulinated peptides (anti-

CCP)

ANA Ro (SSA)/La (SSB)- SLE, Sjogrens

Anti-Smith: largely unused


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Labs in Rheumatology Contd

Anti-ribonuclear protein (RNP)MCTD

Anti-DSDNASLE

Scl70systemic sclerosis, diffuse

Anti-centromeresystemic sclerosis, limited(CREST)

ANCAcANCA antiproteinase 3 vs. pANCA

myeloperoxidase vs. other C3/C4low in autoimmune, high in infection

U/A: proteinura, WBC, RBC, casts


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Imaging in Rheumatology Will likely see some radiology images

Xrays:

Bone (erosions, osteopenia)

Characteristic images:

Pencil-in-cup (psoriatic arthritis)

Gouty tophi

Osteoarthritis

Ankylosing spondylitis

Sacroilitis

CPPD CXR with lung lesions (Wegeners, etc)

MRI/US: better for early erosions, tendinitis,

rotator cuff, meniscus, myositis


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Rheumatological Drugs

NSAIDS: COX1 vs. COX2 inhibitors

Side effects: GI, renal, cardiovascular, platelet dysfunction,

asthma/nasal polyps/allergic rhinitis triad, asceptic meningitis

Not used during 3rd trimesterfetal renal and cardiac

toxicities.

Steroids: Side effects: insomnia, fluid/salt retention, weight gain, HTN,

diabetes, osteoperosis, AVN, myopathy

Use of bisphosphonates rec for >5mg pred daily for >3months

Also use with PPI, calcium/vit D

Plaquenil: antimalarial

Use: SLE, triple therapy for RA, Sjogrens

Side effects: retinopathy, rash, GI


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Methotrexate: dihydrofolate reductase inhibitor

Taken w/ folic acid to reduce side effects

Contraindicated in renal and liver disease

Side effects: GI, mucosal ulcers, hepatotoxicity, b-marrow

suppression, pneumonitis/IPF, hair loss

8wk monitoring LFTs, chem 10, cbc

Sulfasalazine:

use in seronegative spondylarthropathy and RA

Contraindicated with sulfa allergy and G6PD deficiency

Less toxicitysome liver, b-marrow, hemolytic anemia,

neutropenia

Leflunamide: dihydrooratate dehydrogenase inhib

Pyrimidine synthesis inh., alopecia, GI, hepatotox


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Rheumatological Drugs Imuran:

Use in RA, SLE, vasculitis, inflam myopathy Side effects: infection, b-marrow, hepatitis, malignancy

OK to use in pregnancy

Cylcophosphamide: cytotoxic

Use in organ-threatening SLE and vasculitis, SLE nephritis, lung disease in

systemic sclerosis,

IV vs. oral (less side effects with IV)

Side effects: alopecia, GI, infection, b-marrow suppression, infertility (worse

with age), hemorhagic cystitis, bladder cancer

Cbc and u/a monthly

Mycophenalate mofetil:

Use in SLE nephritis, SLE moderate

Side effects: b-marrow suppression, GI


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TNFinhibitors: etanercept, infliximab,adilimumab, golimumab

Use: AS, psoriatic, RA

Best when combined w/ MTX for RA treatment

Side effects: TB reactivation (extrapulmonary),infections, heart failure contraindication,

leukemia/lymphoma, pancytopenia


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Rheumatological Drugs During

Pregnancy

Steroids! (prednisone/solumedrol B) (cleft pallate)

NSAIDS (B,C,D)- not during 3rd trimester (mom bleeding, fetal

cardiac and renal abnormalities)

ASA (C,D) may prevent fetal loss in APS Plaquenil- not classified

MTX (X) -contraindicated! Teratogenicity

Sulfasalazine (B)- ok

Leflunamide (X)- contraindicated, teratogenicity

Azathioprine (D)- ok

Cyclophosphamide (X)- contraindicated, teratogenicity

TNFinh- unknown

IVIG- probably safe


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Raynauds

Cold or fright response-hands/feet- white to blue to

red

Primary vs. secondary

phenomenon (secondary =

worse, ulcerations)

Assoc: SLE,

cryoglobulinemia,

scleroderma (Sjogrens,

RA) vs. Buergers disease

(smoking and

ulceration/amputation of

digits)


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Bursitis

Septic Crystal

Trauma

Dif: cultureand

microscopy

Olecronanon,

trochanteric,subdeltoid,

anserine

Pitting edema


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Monoarticular arthritis

Crystal vs. septic, (also-lyme, gonorrhea, reactive, sickle

cell)

Small or large joint?

Infected = large joint = 50% knee, hip, SI, wrist, elbow,

shoulder, ankle (rarely fingers/toes)

Monoarticular or polyarticular?

Polyarticular is rarely septic.

Fever, serum WBC, uric acid, ESR/CRP = all USELESS

for differentiating between etiologies Recurrent: crystal, lyme, seronegatives

Chronic: mycobacterium, fungi, borrelia,

hypogammaglobulinemia


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Arthrocentesis in Monoarticular

Arthritis

New onset monoarticular arthritis should likely

be tapped

Synovial fluid: cell cnt w/ dif, crystals, culture

and gram stain

Synovial fluid cell cnt tells a lot about etiology

PMNs > 50000 could be cyrstal or septic


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Rheumatoid Arthritis

Epidemiology: F > M, 40-70yo

Pathology:

pannus formation in joint synovium, leads to erosions in bone.

Matrix matolproteinases (MMPs)

Inflammatory cytokines: IL6, TNFalpha, IL1 Synovial disease: angiogenesis, hyperplasia, cytokine release

Clinical: symetric polyarticular inflamatory synovitis

including wrists and MCPs usually

Erosions in 2yrs, long-standing joint disfiguration RF and anti-CCP for prognosis/diagnosis

Treatment: MTX and TNFinh = best treatment

Alternatives: triple therapy (MTX, SSZ, Plaquenil), steroids,

other biologics, leflunomide,


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synovitis

Ulnar deviation

Swan neck

Boutonniere

Physical exam

findings of hands in

RA


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Pannus formation in RA synovium

Vascular formation

Syovial hyperplasia


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RA erosions in various stages


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RA EXTRAARTICULAR -

VASCULTITIS


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RA EYE - SCLERITIS


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RHEUMATOID

NODULE


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SNSAs


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SNSA UNIFYING FEATURES

sacroiliac joint involvement (axial disease) withinflammatory back pain

peripheral joint involvement (non-axial disease)Can be asymetrical and lower extremity

no RF or anti-CCP

extra-articular features:

HLA-B27 +

enthesopathy (tendon insertion site inflam.)

dactylitis (sausage digit)

mucocutaneous disease conjunctivitis/uveitis

cardiac disease (valvular / conduction)

Colitis

urethritis


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SNSAs--uveitis


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SNSAs--enthesitis


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SNSAs--dactylitis


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Circinate balanitis


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Less than 10% of people with HLA B27 will develop SNSA

But 95% of those with AS are B27 positive


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ANKYLOSING SPONDYLITIS

Male, youngB27+

Spine and SI joint involvement

bamboo spine

Inflammatory back pain, AM

stiffnessFusion of spine, increased

fracture

Extrarticular: apical lung

fibrosis, aortitis

Treatment: antiTNF inh


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AS / PULMONARY DISEASE


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SACROILIITIS--early


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SACROILIITISlate ankylosis


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SPONDYLITIS-

SYNDESMOPHYTES in AS


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REITERS / REACTIVE

ARTHRITISReiters Triad: urethritis, uveitis, arthritis

Reactive:1-3wks after bacterial infection (GI/GU)

May be monoarticular, asymetrical, oligoarthritis80% B27 positive

Male predominance

May recur

Bugs: chlamydia, salmonella, shigella, 25% unknown

Tx: NSAIDS, SSZ, MTX, TNFinh


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PSORIATIC ARTHRITISonycholysis DIP arthritis


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PSORIATIC ARTHRITIS


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PSORIATIC ARTHRITIS

Asymetric oligoarthritis

axial disease (spondylitis)

Small joint/RA-like distributionCan proceed skin lesions of psoriasis

RF/CCP negative!

Tx: SSZ, MTX, TNFinhibitor


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IBD-Related/Enteric Arthritis

20% of patients with Crohns/UC

Peripheral (parallels IBD activity)

Axial (does not parellel w/ IBD, more HLA B27+)

Chronic or intermittent, likely asymetric

Worse in patients w/ worse IBD (extraarticular)

Other extraintestinal features

Uveitis

Sclerosing Cholangitis

E. nodosum

Pyoderma gangrenosum

Tx: NOT NSAIDS, either SSZ or TNFinh


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OSTEOARTHRITISEpidemiology:Age-50%>65yrs old, 80% >80yo

Weight/obesity

Trauma

Clinical:Pain (doesnt correlate with xrays)

Heberdens/Bouchards nodes

Possible joint effusionsCrepitus on passive ROM

Imaging:Subchondral sclerosis/cysts

Joint space narrowing, osteophytes

Treatment:Exercise, weight modification, quadriceps

strengthening, shoe inserts

Caspacin cream, tylenol, NSAIDS, COX2inh.,

tramadol, glucosamine, intrarticular hylauronan or

steroids


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NODAL OSTEOARTHRITIS


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OSTEOARTHRITIS

HIP


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EROSIVE OADIP, PIP joints

clinical inflammation


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OSTEOARTHRITIS

HEMOCHROMATOSIS


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OSTEOARTHRITIS

AVN


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SLE


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SLE


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SLE

malar discoid


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SLEJacouds arthropathy


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SLE Treatment and Mortality

Arthralgias/myalgias, rash, fatigue: NSAIDS, plaquenil

Plaquenil: used in almost every SLE pt, steroid-sparing,

cholesterol lowering, anti-thrombotic properties, lowers flares

Severe arthralgias: methotrexate

Severe organ damage, eg. SLE nephritis: cyclophosphamide vs.

MMF, with high dose steroids

Thrombocytopenia: steroids, IVIG

Mortality: PAH, nephritis, accelerated atherosclerosis, other

end organ damage.


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SLE and Pregnancy

Glomerulonephritis, HTN, APS = bad pregnancy outcomes

HELLP/eclampsia/preeclampsia vs. SLE flair

SSA/SSB screening during pregnancy: predicts neonatalSLE/congenital heart block

Treatment of SLE during pregnancy: steroids, plaquenil, imuran.

NO CYCLOPHOSPHAMIDE, MTX, or MMF!


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Drug-Induced SLE Clinically the same except no renal disease

Anti-histone antibody

No anti-Smith or anti-ds dna ab

Complements normal

Drugs: INH, procainamide, hydralazine

Treatment. Short course of NSAIDs orsteroids and DC offending drug


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Antiphospholipid Syndrome

Clinical: Diagnositic:Elevated PT/PTT

Anti-beta2 glycoprotein

Anti-cardiolipin

Increased DRVVT ratio


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LIVEDO RETICULARIS


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MCTD

RA


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Infectious Arthritis:Tuberculous arthritis

Potts Disease: TB of the spinal columnParvovirus: symetric small joint arthritis,

parvovirus IgM +

Lyme

Rheumatic Fever

Monoarticular arthritisHepatitis C

Cryptococcus

HIV


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PARVOVIRUSself resolving


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Septic Arthritis

How? Hematogenous seeding in a damaged (replaced or

diseased) joint

Who? Immunocompromised, RA, elderly, IVDA

Where? Almost always monoarticular, large joint

(knee 50%, rarely fingers or toes)Significant pain on passive ROM

Bugs: S. aureus, strept, salmonella, pseudomonas (IVDA),

pasturella (animal bites)

Arthrocentesis with culture for diagnosis

(culture/gram stain for gonorrhea are usually negative)Treatment: antibiotics (vanco to start unless concerned for gram

negative coverage; surgery pending abx course after repeat

arthrocentesis w/o improvement)


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DISSEMINATED GC

Young, sexually active, usually

knee

Can be migratory arthritis


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CRYSTALLINE ARTHRITIS: Gout

MenMonoarticular flairs vs. chronic

polyarticular

Monosodium urate crystal deposition

in synovium, tendon sheaths, bursae

Inflam: innate immunity, NALP3

inflammasome, IL1

Negative birefringence

Precipiates > 6.7mg/dL

Diet: shellfish, red meat, EtOH

Tophi: soft tissue deposits

CKD = largest risk factor (decreased

excretion of urate)

Drugs: cyclosporin, HCTZ,

furosemide

Diagnosis: crystals


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GOUT


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GOUT


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Gout Treatment

Acute gouty flair:

NSAIDS, colchicine, steroids

Prevention:

1. Daily colchicine: monitor for axonal neuropathy, myopathy,

renal/liver insufficieny, pancytopenia. Interacts withmacrolides, statins, and cyclosporin

2. Uric acid lowering therapy:

Allopurinol (xanthine oxidase inhibition)

Side effects: Stevens Johnson/rash, toxicity in

renal/liver insufficiency Febuxostat (used in renal and liver insufficiency)

Probenecid: increased uric acid renal excretion, measure

urine uric acid first to prevent kidney stones


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CPPDIndolent (pseudoOA, pseudoRA) vs. gouty-like

Age-associated

Positive birefringence

Assoc: acute illness, hyperparathyroid, Wilsons, hemachromatosis

Treatment: like gout, but not uric acid lowering


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Systemic Sclerosis (Scleroderma)Connective tissue disease, fibroblast activation, microvascular injury

Diagnosis: skin thickening and sclerodactyly are the hallmarks

Classification:

1. Limited Systemic Sclerosis (CREST)

Calcinosis, Raynauds, esophageal dysmotility, sclerodactyly,

telangiectasias

Skin thickening only distal to elbow/knee, +/- face

2. Diffuse Systemic Sclerosis

Skin thickening proximal to elbow/knee, +/- face

Skin: thickening, flexion contractures, calcinosis, ulcers, telangiectasias

Raynauds (95%)MSKL: palpable tendon friction rubs, inflammatory arthritis, inflam

myopathy overlaps

GI: all scleroderma has some GI invovlementesophageal most

common (GERD, Barrets, strictures), illeus, pseudoobstruction


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Systemic Sclerosis (Scleroderma)

Pulmonary: high mortality!1. ILD (diffuse sclerodermabibasilar ground glass, reticulolinear

scarring)

2. PAH (limited scleroderma)

Renal: scleroderma renal crisis (diffuse scleroderma)

Characterized by severe HTN, hemalytic anemia, low plts,

proteinuria and renal failure

Treatment: rapid uptitration of ACEinhibitors

Risk: prednisone treatment of scleroderma

Systemic Sclerosis sine scleroderma: characteristic organ involvement

without associated skin thickening


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MORPHEA & LINEAR

SCLERODERMA (isolated skinthickening)


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CREST


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PSS SKIN


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SCLERODERMA GI


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SCLERODERMA

ANTIBODIES


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SCLERODERMA LUNG

POLYMYOSITIS / DERMATOMYOSITIS


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POLYMYOSITIS / DERMATOMYOSITIS

Clinically: symetric proximal muscle weakness, +/- esophageal, neck

and/or respt muscle weakness

ILD association with either

Labs: elevated CK most sensitive (also aldolase, CKMB, AST/ALT)

Usually +ANA

Anti-Jo1: 20% pts, anti-histidyl tRNA synthetase (assoc. with ILD,

polyarthritis and Raynauds)

Diagnosis: clinical suspicion with elevated CK, EMG and MRI findings

of weak proximal muscles, biopsy = gold standard

Treatment: high dose steroids until CK decreases, then MTX or steroid-

spairring agent. Cylcophsphamide for ILD, IVIG may workDermatomyositis: association with age-appropriate cancer, ovarian

cancer


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DM

Shawl sign heliotrope


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GOTTRONS (DM)


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IBM

Vasculitis


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VasculitisLarge Vessel Vasculitis:

Giant Cell Arteritis/PMRTakayasus arteritis

Medium Vessel Vasculitis:

Kawasakis

PAN

Small Vessel Vasculitis:

Wegeners

Churg-Strauss

MPAHSP

Cyroglobulinemic

Cutaneous leukocytoclastic

Giant Cell Arteritis


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Giant Cell Arteritis

Granulomatous vasculitis

Extracranial branches of the internal carotid

Women >50yo

Clinical: headache, jaw claudication,

systemic symptoms, fever, visual

disturbance, tenderness of temporal artery

Diagnosis: clinical features + ESR = biopsy

High dose steroids/ASA if suspect, b/fbiopsy to prevent blindness/stroke

Long steroid course (2yrs plus), relapsing

PMR


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PMR

Acheing and morning stiffness in pelvic andshoulder girdles, in conjunction with high ESR

Old white people

40-50% of pts with GCA have PMR, only 10% ofPMR pts have GCA

Clinical/lab diagnosis

Diagnostic and therapeutic: rapid response to 10-20mg of prednisone

Takayasus Arteritis


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Takayasu s Arteritis

Granulomatous branches of aorta and itsbranches and the pulmonary arteries

Young women

Systemic constitutional symptoms, HTN,asmetrical limb blood pressure, symptoms

of occlusion (stroke, vision changes, etc)

Dx: arteriography

Tx: MTX and prednisone

Chronic illness, relapse, some w/o

remission


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TAKAYASUS

P l t iti d


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Polyarteritis nodosa

Necrotizing inflammation of medium-small

arteries without glomerulonephritis

Clinical: hypertension, fever, mskl symptoms,

vasculitis of GI, heart and renal arteries

Dx: arteriography of visceral/abdominalarteries: microaneurysms, stenosis, string of

beads

Tx: cylcophosphamide and steroids forinvolvement of GI, heart, or CNS

No relapse


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POLYARTERITIS NODOSA


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Wegeners Granulomatosis

Necrotizing granulomatous vasculitis

Lung nodules, sinus/cartilage damage,

glomerulonephritis

Dx: biopsy, usually VATS, of lung tissue

focal/ semgental necrotising

glomerulonephritris, pauci-immune.

C-ANCA anti-PR3 positive

Tx: cyclophosphamide/high dose steroids


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WEGENERS

Microscopic Polyangiitis


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Microscopic Polyangiitis

Small vessel necrotising, non-granulomatousvasculitis

Clinical: glomerulonephritis, pulmonary

hemorrhage, mononeuritis multiplex, cutaneoussmall vessel vasculitis with palpable purpura,

fever

Dx: biopsy, pauci-immune

P-ANCA, MPO positive

Tx: cyclophosphamide, high dose prednisone

ANCA


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ANCA

C-ANCA (PR3)-Wegeners

P-ANCA (MPO)-CSS/MPA

Churg Strauss Syndrome


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Churg-Strauss Syndrome

Clinical: asthma, fever, hyper-eosinophilia, and

vasculitis

Vasculitis of many different organs possible

Some have c or p-anca

Tx: prednisone (unless life threatening =

cyclophosphamide)


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Other small vessel vasculitides

HSP

Cyroglobulinemic

Cutaneous leukocytoclastic

Drug-induced


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HYPERSENSITIVITY

VASCULITIS / HSP


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CRYOGLOBULINEMIA

FIBROMYALGIA


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FIBROMYALGIA

MSKL pain with palpation, without evidence of arthritis

Treatment: antidepressants, exercise, OSA treatment


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HIV

Incomplete Reiters

PsA

RA goes away..

Myopathies


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SARCOIDOSIS


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REFLEX SYMPATHETIC

DYSTROPHY


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REFLEX SYMPATHETIC

DYSTROPHY


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Sjogrens Syndrome

Sicca complex

Keratoconjunctivitis (lacrimal gland)

Oral sicca (parotid)

Lymphocytic infiltrate (CD4)

SSA (Ro), SSB (La), RF, ANA, polyclonalgammopathy

Woman age 30-50

Extraglandular ILD, PBC, vasculitis, neuropathy, arthritis, Raynauds, fever,

fatigue, adenopathy/lymphoma, Hashimotos, myositis

Primary vs secondary (RA)


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BEHCETS


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Relapsing Polychondritis

Inflammation and destruction of cartilage

structures (ears, nose,throat)

Auricular/ear pain and swelling, califlowerear, saddle nose deformity, upper

respiratory cartilage destruction

NSAIDs, dapson, or steroids

Adult Stills Disease


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Major Criteria:

Fever >39

Arthralgias/arthritis (synovitis)

Salmon-pink macular rash on trunk/extrem

Lekuocytosis, prominent left shift

Minor Criteria:

Sore throat, LAD, elev LDH, AST/ALT, negative

ANA and RFTreatment: dependsusually NSAIDs and ASA for

minor symptoms

abim logy review 2010

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