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Rheumatology Boards ABIM

Terms in this set (119)

ANA fluorescence importance
useless except centromere staining - (limited scleroderma (CREST), primary biliary cirrhosis)
ANA frequency in dz
Drug induced SLE - 100%
SLE - 90%
Polymyositis/dermatomyositis - 40-60%
RA - 40%
MCTD - 95%
Limited scleroderma and diffuse scleroderma - 60-90%
Sjogrens - 70%
Nl population - 30% (1:40), 5% (1:160)
So, need more specific subtype testing if positive
ANA subtypes
Anti-dsDNA - specific for SLE, can be + if taking TNF inhibs. Marker for dz activity & potential for renal dz
Anti-Sm - specific for SLE, often seen with U1-RNP
Anti U1-RNP - sensitive, not specific for MCTP (neg rules it out)
Anti-histone - sens, not spec for drug induced SLE (procainamide, hydralazine, chlorpromazine, quinidine)
Anti-Scl-70 - diffuse scleroderma, increased chance of interstitial lung dz
Anti-centromere - limited scleroderma; increased Pulm HTN
Anti-synthetase (Jo-1) - myositis; increased ILD
Anti-SSA, SSB tests
SSA (Ro) - SLE, neonatal SLE, Sjogrens, myositis; not in scleroderma; maternal transfer to baby - neonatal heart block
SSB (La) - SLE, Sjogrens; sometimes found with SSA; can see maternal transfer to baby
ANCA testing
Get p & c-ANCA. If positive, check ELISA for subtypes anti-PR3, anti-MPO
c-ANCA + anti-PR3 - Wegeners
p-ANCA, MPO positive - Churg-Strauss, PAN, pauci-immune glomerulonephritis, microscopic polyangiitis, anti-glomerular basement membrane dz
p-ANCA, MPO negative - Crohns, UC, chronic active hepatitis, PBC, PSC, PAN, chronic arthridities
Compliment tests
low in active SLE, vasculitis
Can also be low from genetic deficiency, consumed with activation, or underproduced (HELLP syndrome)
C3 - consumed with any compliment activation
C4 - consumed with only classic pathway activation
CH50 - looks at total hemolytic component of classical pathway
Rheumatoid factor and anti-CCP (citrullinated cyclic peptide)
RF - pos in 80-85% of RA, not specific
CCP - appears earlier, greater specificity
HLA's
HLA-B27 - 7-8% of nl population; 60-80% in Reiters + reactive spondyloarthropathy; 90% with ank spondy; 60% of sponylitis pts with psoriasis or IBD are positive; 80% in Yersinia, Salmonella, Shigella arthropathy
HLA-DR2, DR3 - Sjogrens, polymyositis
HLA-DR4 - severe RA
Joint fluid evaluation
Normal - 0-200 WBC
Non-inflammatory- 200-2000 WBC - OA, neuropathic joins, hypertrophic osteoarthropathy, occasionally SLE, scleroderma, rheumatic fever
Inflammatory - 2K-50K WBC - RA, SLE, SS, crystals, ank spon, IBD-assc arthritis
Septic - 50K+ - >75% neurophils. Occasionally see this many WBC with crystals
Crystals in joint
Gout - yellow, negatively birefringent
CPPD - blue crystals, positively birefringent
To be sure they are causing the inflammation, you should see them intracellularly
Collagen vascular diseases
Marfan syndrome - long limbs, aortic root dilation, ectopia lentis
Ehler-Danlos - skin elasticity and joint hypermobility; types - classic (most severe, easily scarred skin and hypermobile joints), Hypermobile (mainly joint symptoms), vascular (mainly skin issues and rupture of large vessels)
Osteogenesis imperfecta - procollagen gene defects; osteopenia, brittle bones, blue sclera, teeth problem, hearing loss
Pseudoxanthoma elasticum - autosomal recessive; affects skin, blood vessels, eyes. Angioid steaks on fundoscopy; recurrent GI bleeding
Prevalence of RA
1% overall, 40-50 yo, female: male 3:1, 10% with genetics
Labs for RA
RF - 80-85%
anti-CCP - 97%, earlier than RF; assc with erosive RA
HLA-DR4 - aggressive RA
Joint fluid - inflammatory (2K+wbc), decreased viscosity; finf IL-1, IL-6 in joint fluid
Markers for severe course in RA
high titer RF
positive anti-CCP, HLA-DR4,
Constitutional symptoms
Insidious onset
early XR evidence of erosive dz
extraarticular dz
Diagnostic criteria for RA
4+ of the following:
AM stiffness > 1 hr for 6 wks
Swelling of wrists, MCP, PIP x 6 wks
Swelling of 3 joints x 6 wks
Symmetric joint swelling x 6 wks
Rheumatoid nodules
Erosive synovitis XR changes in hands
Positive RF
Causes of symmetric, polyarticular hand arthritis
RA
SLE
scleroderma
psoriasis
Hemochromatosis vs. RA
both in MCP, PIP
HC is not symmetric
HC has hook-like osteophytes and chondrocalcinosis - not in RA
Other joints in RA outside hands
Cricoarytenoid joint, TMJ - hoarseness, sore throat, neck pain
Knee - most common single joint
Can see carpal tunnel, tarsal tunnel
C-spine - with chronic, sever dz get C1-2 subluxation (>3mm, best on lateral flex/ext XR); recurrent HA starting at base of skull, arm tingling, dizziness; no surgery unless sx; higher risk for cord damage with trauma from intubation-syncope, lightheaded, nystagmus post-op: do imaging in any chronic RA pre-op
T/L spine - usually spared
Extraarticular manifestations of RA
Cardiac - pericarditis, myocarditis, rhem nodules on valves, CAD (3x increased risk)
Renal - drug related, amyloid late
Lung - pleural effusion (low ph, glu); diffuse fibrosis, intrapulmonary nodules
Vasculitis - like polyarteritis nodosa (nail fold infarcts), necrosis/ ulceration over malleoli
Nerves - mononeuritis multiplex, carpal tunnel, cervical myelopathy
Eyes - episcleritis, scleritis, sicca
Skin - rheum nodules in 25% (indicate more severe dz)
Blood - anemia of CD; neutropenia
Triad in Felty syndrome
RA, splenomegaly, neutropenia
Usually with skin nodules, increase risk of death from infection
Rx - MTX, cyclosporin A, steroids, granulocyte CSF; may need splenectomy - if fails to control, poor prognosis
Large granular lymphocyte syndrome
ALSO HAS - RA, splenomegaly, neutropenia (like Felty);
differences: LGL less associated with RA, progresses to LGL leukemia, do poorly with splenectomy
Drug groups for tx of RA
1. NSAIDs - non-selective & COX-2 inhibitors
2. Non-biologic DMARDs - MTX, leflunmide, hydrochloroquine, sulfasalazine, gold
3. Immunosuppresants - azathioprine, chlorambucil, steroids, cyclophosphamide, cyclosporin, mycophenalate, tacrolimus
4. Biologics - TNF inhib(monoclonal Abs, soluble receptors), IL-1, IL-6 antaganosts, anti-B cell antibody, T-cell inhibitors
5. Misc - acetaminophen, colchicine, dapsone, IVIG, plasmapheresis/exchange, thalidomide, intraarticular viscosupplimentation
Why treat RA?
70% of active, polyarticular, RF pos dz develop joint erosions and damage in 2 yrs, can drastically reduce with early aggressive tx
Treat aggressively those predicted to get erosive dz
Indications for early DMARD treatment in RA
Constitutional sx, progressive synovitis, rhem nodules, vasculitis, extraarticular manifestations, high ESR, high RF, joint space loss or erosions on XR, HLA-DR4 positive; not using anti-CCP yet, although predicts future erosive dz
Steps of tx in RA
NSAIDs +/- DMARD ->add steroids -> add biologics -> add other
NSAID's added to DMARDs mainly to help the pain
NSAIDs in RA
do not alter course of disease, used for pain control
Celecoxib - presumed decrease GI effect, not studied, less platelet effect; use with great caution in pts at CAD risk - increases risk for adverse CV events dose related
Interact with Lithium, oral hypoglycemics
Increase BP
DMARDs
slow onset (months)
minimal antiinflammatory effect
If any cost issues, use SSZ, HCQ if mild disease
Methotrexate
Most predictable effect, usually best tolerated - use as initial treatment!
Contraindicated with renal or liver dz, pregnancy
Side effects - alopecia, GI (N/V/D, mucositis), marrow suppression (give folate to prevent), renal failure
Can cause idiosyncratic pneumonitis
Monitor LFT, albumin, Cr, CBC q6-8 wks
PFT's if sx of dyspnea
Leflunomide (Arava)
pyrimidine antagonist
Initial tx in RA if cannot tolerate MTX
Same side effects as MTX except don't need folate
Screen for TB before use
Avoid in pregnancy
Hydroxychloroquine
Need annual opth exam for retinopathy
Avoid with renal issues - increase risk of eye probs
Monitor muscle strength (myopathy)
Can exacerbate psoriasis rash
Sulfasalazine
Also used in UC
Side effects: sulfa allergy, N/V/D, abd pain, oligospermia, cytopenia, increased transaminases
Gold
IM only, 50-75% response
Can cause rash, stomatitis, proteinuria, leukocytopenia
DC if pruritis, mod/severe stomatitis, proteinuria >500mg/24hr, WBC<3000, plt<100K
D-penicillamine
side effects like gold+pneumonitis, myasthenia gravis, dysgeusia
Biologic agents
TNF inhib monoclonal abs -Infliximab, adalimubab, certolizumab, golimbumab, etanercept- used in RA, IBD, psoriasis, ank spon; most beneficial combined with MTX; side effect of URIs
IL antagonists (Anakinra -IL-1, tocilizumab IL-6) use if RA refactory to MTX/TNFI
Anti-CD20 ab - rituximab- use with MTX if refractory to TNFI
T-cell inhib - abatacept- use with refractory joint dz
Side effects of biologics
All - increased risk of serious infections, esp fungal & TB
Most common SE - URI's, can worsen psoriasis, reactivate Hep B
No live virus vaccines while using, annual TB screening
Infliximab - increase risk of heme malignancies
Rituximab - mucocutanous issues (stevens-johnson, toxic epidermal necrolysis),PML, tumor lysis syndr, infusion rxns (HoTN, bronchospasm ,ARDS, MI)
Seronegative spondyloarthropathies
Include: ank spond, reactive spondyloarthopathy (reiters), psoriatic arthritis, enteropathic arthopathy with IBD
Predilection for spine, SI joints, ensethopathy (in fingers causes "sausage digits" - if with pitted nails as well, it is psoriasis)
Get asymmetric oligoarthritis, esp in lower extr
Often with HLA-B27 assoc
Ankylosing spondylitis
Marked spinal, SI joint fusion ("bamboo spine"); pain better with exercise; often with painful sacroileitis
Onset in young adults, teens
Systemic - uveitis (30%), aortitis (3%) (AoR, CHF, heart block), apical pulmonary stenosis
Dx - XR signs of symmetric sacroileitis w/o IBD, psoriasis. Don't need HLA testing
Tx - back training, exercises; DMARDs and biologics to prevent joint fusion, pain mgt with NSAIDs
Reactive arthritis
Most common cause of non-traumatic arthritis under 50yo
Usually a reaction to infection elsewhere, immune rxn
Ask about recent infections, esp diarrhea, urethritis/STD, viral infections
Evaluate conjunctiva, urethra for other types of spondyloarthropathy
Rx - NSAIDs, treat underlying infection
If refractory, use MTX, SSZ
Reactive sponyloarthropathy (Reiters)
Triad of urethritis, conjunctivitis, asymmetric arthritis (usually large LE joints)
Skin lesions - keratoderma blenorrhagicum (looks like pustular psoriasis), circinate balanitis, oral lesions
Infections causing it: GU (chlamydia, ureoplasma), GI (salmonella, shigella, yersinia, campylobacter), C. diff, C. pneumoniae, HIV
Psoriatic arthritis
Nail findings in 20-30% - pitting, onycholysis, "oil spots" under nails
Can develop arthritis mutilans - severe resorptive degeneration of joints
Affects DIP joints with nail issues
Tx - NSAIDs, add DMARDs if ineffective; biologics also help
Avoid HCQ, B-blockers - can exacerbate skin dz
Hand joints affected by which dzs
DIP - psoriatic, OA (hebredens nodes)
PIP - OA (bouchards nodes), RA, SLE
MCP - RA, SLE, hemochromatosis
OA causes
Idiopathic
Inflammatory - gout, CPPD, hemochromatosis, DM neuropathy
Trauma
Exam findings in OA
slowly progressive, worse with activity
Usually hands, feet, knees, hips, spine; rare in ankles, wrist, elbows
Hebredens, Bouchards nodes
XR - synovitis, asymmetry, no calcium deposits (chondrocalcinosis)
Dx of hand OA
3 of 4 criteria:
1. bony enlargement in 2 or more: DIP/PIP, 1+ MCP
2. Bony enlargement of 2+ DIP
3. Fewer than 3 MCP swellings
4. Deformity of any DIP/PIP
Features of knee OA
Pain superior to patella, deep inside joint (medial and inferior is usually anserine bursitis)
Age >50
AM stiffness <30 min
Bony enlargements
Crepitus
Noninflammatory synovial fluid
ESR<20
Bone spurs on XR
Gout causes
Increased production (10%) - idiopathic, leukemia, hemolysis, psoriasis,exercise, fructose ingestion, G6PD deficiency
Decreased excretion (90%) - idiopathic, CKD, lead nephropathy, alcohol, drugs, DKA)
Differentiate with 24hr urine uric acid level - nl in underexcreters, high (>800) in overproducers
Note: diuretics and nicotinic acid increase uric acid levels
Treatment of chronic gout
Probenicid (increases excretion) for underexcreters
Allopurinol or febuxostat in overproducers, can use in underexcreters as well
Avoid probenicid in >60, tophi, Cr>2, history of UA stone, overproducers, chemo prophylaxis
No not start, stop or change dose in an acute flare!
Gout pearls
low doses ASA interferes with UA excretion, high dose, improved it
Can cause fever
rare in postmenopause; see as new onset, rheumatoid like in older lady
joint edge erosion from tophi
Never see in pts with RA
DDX acute monoarticular joint swelling
crystals, infection, or fx/trauma
Calcium pyrophosphate dihydrate dz (CPPD)
Usually idiopathic, >60
Usually underlying joint damage
If <50yo, look for primary hyperparathyroidism, hypothyroidism, hemochromatosis
Affects wrists, MCPs, shoulder, elbow, ankles - not joints usually seen with OA
Blunted, rhomboid, blue, positive direfringence
Tx - aspiration, NSAIDs, intraarticular steroids
Hydroxyapatite arthropathy
Idiopathic, hypercalcemic, hyperparathyroid states, in dialysis pts
Elderly
Very destructive, shoulders, hips, knees, hands
Noninflammatory fluid, no visible crystals - very small, nonbirefringent, need electron microscope or special stains to see
Septic arthritis pearls
S. aureus is usual cause, esp in RA
Adolescents and young adults - suspect gonorrhea
Sickle cell - salmonella, pneumococcus
Human bites - anaerobes, eikenella
Animal bites - pasturella
Extensive comorbidities - GN's, group a strep, pneumococcus
Indolent, chronic, non-inflammatory +/- bloody - TB, mycobacterial, fungus
IVDU - staph, strep, GN's, esp pseudomonas
Prosthetic joints - S. aureus, coag neg staph; "loosened prosthesis"
Gonococcal arthritis
Risks - pregnancy, menstruation
Sx - fever, migratory polyarthralgis, tenosynovitis, red papules that become pustular
Joint fluid - 10-20K WBC, sterile, bloody in 65%
Culture all possible mucosal surface directly to Thayer-Martin chocolate agar
Nongonococcal septic arthritis
Gram stain usually positive
Treat based on gram stain and risks
Anti-pseudomonals if suspect IVDU
Treat chlamydia if have gonococcal
Whipple disease
PAS-positive macrophages in intestinal lamina propria, mesenteric nodes. Arthralgias (intermittent, inflammatory), cardiac, and neurologic (slowly progressive dementia) symptoms are common, constitutional sx, fever. Most often occurs in older men. Due to Tropheryma whippleii. Rx - Bactrim DS x 1 yr
Tuberculous arthritis
affects weight bearing joints, knees. monoarticular. usually a complication of dissemination. Mildly symptomatic, chronic effusion persisting for years
Dx - bx of synovium or TB PCR
Treat like pulmonary TB
Viral arthritis
Parvo B19 - slapped cheek, recent exposure to sick kids
Acute rheumatic fever - fever, polyarthritis, chorea, erythema marginatum, nodules, evidence of carditis
Lyme arthritis
Part of stage III dz, sometime stages I+II asymptomatic
Few months to 1-2 yr post bite in 50% of untreated pts
Asymmetric arthritis, one or more large joints.
Sx wax and wane
Dx with ELISA; false pos from SLE, RA, RMSF, syphilis; confirm with western blot
Adult Still disease
Vanishing salmon pink macular rash, appears and disappears with high spiking daily fevers
Mild oligoarthritis, mildly inflammatory fluid
Sore throat, serositis, adenopathy, myalgias, arthralgias; can progress to destructive polyarthritis, joints can actually fuse
Labs-anemia of CD, polycythemia, low titer ANA, increased ESR,CRP,ferritin, transaminases
Rx- like RA, but use MTX, SSZ, NSAID with caution in liver dz
Hemochromatosis arthritis
20-40% of HC Pts, usually after 50
small joints first (2nd and 3rd MCP), then bigger joints (knees, ankles, shoulders)
Joint fluid is noninflammatory, helps MCP joint dz from inflammatory ones
AM stiffness <30 min
XR narrowed joint space
See CPPD deposits in 50% of Pts.
Acromegaly arthritis
Pituitary secreting tumor, excess growth hormone
Cartilaginous expansion, affects big bones and hands
XR looks like OA
Tissue enlargement in hands and feet
See CPPD, carpal tunnel with it
Dx- IGF-1 screening first
Neuropathic joints
Due to repeated trauma from loss of pain sensation, proprioception
Leads to severe OA, can see erosions, bony fragments - body tries to repair, becomes overgrown with noninflammatory effusions
Look for horrible bones, but painless
Hypertrophic pulmonary osteoarthropathy
Joint effusions in smoker with clubbing
Assc with lung malignancies
Periosteal bone formation with noninflammatory joint effusions, clubbing
XR - periosteal bone growth adjacent to radiolucencies
SLE risks
Female >male, African American, oral contraceptives, estrogen replacement
Joint lesions in SLE
Symmetric, non erosive polyarthritis of small joints. RA is erosive
Skin lesions in SLE
Discoid - hyper pigmented edges, light centers
Systemic - UV sensitive, butterfly rash, spares nasolabial fold
Subacute cutaneous - UV sensitivity causing psoriatic like lesions
Can also see alopecia, non-painful apthous ulcers on nasopharynx
Other systems in SLE
Lungs-pleuritic CP, maybe effusion, alveolar infiltrates, pneumonitis with fibrosis, pulm HTN
Heart - pericarditis, myocarditis, valve lesions (libman-sacks endocarditis- culture neg, from antiphospholipid ab), increased risk of CAD
Kidneys - major cause of mortality, assc with anti-dsDNA, all degrees if disease
Blood- anemia CD, leukopenia, thrOmbosis from APLAS, dooms pos hemolytic aneMia, TTP
CNS- many possible lesions, clear with dz tx.
Labs in SLE
ANA, anti-dsDNA, antiSm
Low complement
Dz of SLE
Need 4 of 11:
Rash with UV exposure, malar rash, oral or nasopharyngeal ulcers, discoid rash, arthritis (symmetric, non erosive), serositis, renal involvement, hematologic changes, neuropsych features, anti-dsDNA, sm, or APLAS, positive ANA
SLE with pregnancy
Higher risk of miscarriage. ESP if dsDNA, APLAS positive
Prognosis in SLE
Worse if elevated dsDNA, increas risk of nephritis
Drug induced SLE
Procainaminde,hydralazine, chlorpromazine, PTU, minocycline, anti-tnf drugs
Usually no kidney or lung involvement
ANA, anti-histone positive
MCTD
Signs of several disease mixed. Good response to steroids, good prognosis
Anti-u1-RNP positive
Antiphospholipid antibody syndrome
Includes lupus anticoagulant, anticardiolipin antibody, false-positive VDRL/RPR
Do not need to have SLE, 50% are primary
Labs- nl PT, prolonged PTT not corrected with mixing plasma, prolonged plasma clot time
Hyper coagulable, clotting studies in vitro effect only
S/sz-recurrent thrombosis, spontaneous miscarriages, young with CVA/TIA, valve abnormalities, livido reticularis, thrombocytopenia
Tx-lifetime anticoagulation if any clots
Sjogren syndrome
CD4 infiltrate in lacrimal, salivary glands, autoimmune
Elderly present with sicca syndrome-dry eyes, dry mouth
Other sx - can see parotitis, adenopathy, purpura, interstitial nephritis
Labs-90%RF+, 70%ANA+, 50% SSA(Ro)+, 30% SSB(La)+
Can be secondary to any rheum dz
40x increase risk of lymphoma!!!!
Dx - sxs, autoantibodies, bx, schirmer test
Rx - symptomatic unless systemic sxs
Types of Scleroderma
Scleroderma=thickened hard skin
Systemic sclerosis (SSc)=scleroderma + organ involvement
Diffuse - multiorgan involvement; 30% are anti-topoisomerase antibody positive, which is assc with interstitial lung dz, decreased survival
Limited SSc - CREST sxs; 10% anti-centromere ab +, which causes pulm HTN
Skin manifestations in SSc
Limited - distal extremities, face, telangiectasias
Diffuse - entire extremities, chest, abdomen, face, less likely to have telangiectasias
Abnormal nailfold capillaries - more abnormality=worse severity of dz
Joint manifestations of SSc
mild symmetric stiffness +/- synovitis, rarely involved hands; tendon friction rubs, esp at elbows - pathognomonic for SSc
Raynauds in SSc
Colors - white->blue->red
Both limited and diffuse type
can see fingertip ulcerations - DO NOT see the ulcerations in primary Raynauds, so some underlying rheum abnormality is present (can see in almost all rheum d/os)
If no Raynauds, it's likely not SSc
Rx - CCBs, ACE/ARB, ASA
Muscles in SSc
mild muscle pain, mild weakness, mild CK elevation
Due to atrophy from skin tightness
Lungs in SSc
Main cause of death in SSc!!!!
Limited - intimal proliferation with no alveolar inflammation->pulm HTN; esp. in anti-centromere positive; usual Pulm HTN rx
Diffuse - alveolitis, pulmonary fibrosis, interstitial lung dz, esp anti-topoisomerase positive; Rx with cyclophosphamide
Kidneys in SSc
Diffuse only!!!!
renal crisis - acute HTN, ARF, active sediment in young female (suspect SSc if present)
Can see thrombocytopenia - like TTP
Rx - ACE
Steroids can precipitate this, avoid in diffuse SSc!!!
GI in SSc
Diffuse - wide mouthed diverticuli
Limited - watermelon stomach, telangiectasias, iron def anemia
Both - dysmotility, esp esophagus and stomach; LES relaxation - severe GERD
Primary biliary cirrhois in 15% (anti-mitochondrial antibody positive)
Heart in SSc
cor pulmonale, restrictive pericarditis, conducion defects
Rx for SSc
No medicines change the course of disease!!!
Sx treatment for specific organs
Avoid steroids - can precipitate renal crisis
Can use D-penacillamine for skin disease, but side effects limit use
Diffuse SSc peak in 1st 5 years, so no further organ involvement after that, although affect organs continue to deteriorate
Eosinophilic Faciitis
Sclerodema like skin changes, spares hands
Often follows rigorous exercise!!! can be paraneoplastic
Tender migrating edema
Keys - NO Raynauds, follows exercise, nl nailfold capillaries
Peau d'orange skin in prox forearms, upper legs
Labs - peripheral eosinophilia, elevated ESR, nl ANA, RF; bx with eosinophilic infiltrate
Rx - usually self limited, mod dose steroids or MTX
Raynaud phenomenon
Primary - young women, usually within a few years of menarche
Secondary - due to CTD - SSc, RA, SLE, SS, MCTD, occ dermatomyositis
Suspect secondary if: male, >40, asymmetric, fingertip ulcerations, coexistant autoimmune or vascular dz
Polymyositis
proximal weakness, mild myalgias, proximal legs first
No neuropathy
Minimal to no pain - distinguished from FMS
Dx - abnormal, myopathic EMG, increased CK, abnormal muscle bx (cytotoxic T-cell infiltrate)
can progress to dysphonia, dyspnea, eKG changes
Dermatomyositis
Like polymyositis, but with skin involvement
Skin only - amyopathyc dermatomyositis
Skin changes - mod/deep purple/red papules, scaly, photosensitive rash in face and neck (shawl sign, V neck) and extensor surface of joints; heliotrope rash (violet, affects upper eyelids-not seen in SLE, can affect cheeks, forehead), Gottron's papules (flat, red scaling on knuckles of MCPs
Path- perivascular inflammation B cells and CD4T
Groupings for Polymyositis & dermatomyositis
1. Primary idiopathic polymyositis
2. Primary idiopathic dermatomyositis
3. PM/DM asc. with malignancy
4. PM/DM in childhood
5. PM/DM assc with CTD
Autoantibody associations with PM/DM
1. Anti-Jo - rapidly progressing lung dz, "mechanic's hands", arthritis and Raynauds
2. Anti-Mi2- 20% with DM; good prognosis
3. Anti-SRP - severe, aggressive PM
4. Anti-PM-Scl and Anti-Ku - scleroerma-myositis overlap syndrome
Ro, La, RNP, Sm suggest myositis assc, not primary PM/DM
Cancer and PM/DM
present in 7-10%PM, 10-15% DM
Risk increased with age
Any pt over 50 with new dx PM/DM needs age-appropriate cancer screening (not full body CT)
Failure to respond to tx, rescreen for CA
Treatment for PM/DM
High dose steroids - 80% respond within 6 wks
Antimalarials for rash in Dm
steroid resistant - try mTX, azathioprine, cyclosporin A
Inclusion body myositis
Most common inflammatory myopathy >50yo, esp men
Distal weakness (weak handshake), can see dysphagia
look for drug exposures - antimalarials, steroids, colchicine, statins, alcohol) to look for other causes
No lab help, only muscle bx - vacuoles and fiber inclusions
Rx - remove precipitating drugs, high dose steroids
Minimally responsive to treatment
Colchicine myopathy/neuropathy
mimics PM with proximal weakness
Key is neuropathy findings - don't see in PM- paresthesias
Drug induced myopathy
Lipid lowering druds, steroids
Steroids - weak, muscle pain but nl CK/EMG; better with rehab, DC drug
Fibromyalgia
neurotransmitter disorder
Women:men 10:1
Sx- diffuse muscle aches, stiffness, fatigue, non-restorative sleep
Exam - 11+ tender points (control points that shouldn't be positive - forehead, digits, anterior thigh)
can complicate any chronic illness - check thyroid, viral hep, vit d, OSA)
Tx - stress reduction, exercise, sleep restoration, antidepressants, antiepileptics, non-narcotics
myofascial pain syndrome
localized pain site, may be assc with trauma
local anesthetic injection to trigger point sometimes helps
Complex regional pain syndrome (prior RSD)
H/O trauma to extremity, can see shoulder-hand syndrome post-MI
sx-severe pain, tenderness, touch sensitivity, later tight, cool, shiny skin; flexion contractures; can get profound osteopenia
rx - NSAID's, pain modifiers, short course steroids, early aggressive therapy
Vasculitis overview
Constitutional sx big - myalgias, arthralgias, neuropathy, fever, malaise, wt loss
Think about with palpable purpura
Dx with bx of the most affected tissue if able
Giant cell arteritis (GCA)
Affects head + neck, >50 yo, F>M
Sx - temporal HA, diplopia, amaurosis fugax, scalp tenderness, jaw claudication; 40-50% get ischemic optic neuropathy if untreated (blindness)
If extracranial sx, worse risk of thoracic aortiitis
Dx - temporal art. bx within 10 days of starting steroids, bilat is better, negative doeas't exclude
Rx -high dose steroids for months, very slow taper
50% have PMR - body pain+vision sx+ESR in elderly=PMR+GCA
Polymyalgia rheumatica
20% also have GCA, 50% of PCA have PMR
Elderly, profound AM stiffness, shoulder girdle+hips ache, hand swelling (no weakness, unlike PM/DM)
Labs - nl CK, elevated ESR
Rx - great improvement with low dose steroids, slowly taper over 1-2 yrs - if no dramatic response, think of other dx (GCA, Cancer)
Takayasu arteritis
Pulseless disease, young women, esp asian
Sx - initial FUO, then 1-2 yr later claudication, renal art stenosis, raynauds, erythema nodosum
Dx - angiography or MRA
Rx - steroids, CCBs for vasospasm; anticoagulation if very stenotic vessels; DMARDs for steroid sparing
Tends to recur, guarded px
Aortiitis
Assc with syphilis, endocarditis with mycotic vegetations, GCA, spondyloarthropathies
In syphilis - tertiary manifestation, rarely see dissection or rupture
Polyarteritis Nodosa (PAN)
Assc with Hep B; CMV, RA, HIV, Hodgkins, hairy cell leukemia, allergies give PAN-like vasculitis, but not true PAN
Affects almost all organs EXCEPT lungs (no pulm hemorrhage)
Sx - diffuse- fever, anorexia, wt loss, arthralgias, mononeuritis multiplex, CNS sx, abd sx (mesenteric adenitis), rashes (palpable purpura, levido reticularis), foot drop
dx - angiogram-diffuse small saccular aneurysms, bx if peripheral
Rx - treat HBV, prednisone+cyclophosphamide;10-20% survive 5yrs if not treated
Churg-Strauss vasculitis
Necrotizing pulmonary-renal vasculitis with eosinophilic granulomas
Always have hx of asthma+/- sinus dz/ allergies
lab- periph eosinophilia, p-ANCA, anti-MPO;
Tx - steroids, cyclophosphamide
Wegener's Granulomatosis
Also necrotizing granulomas in sinuses, lungs, kidneys; occasional rashes, ulcerations, migratory large joint arthritis
Bx -sinus, low yield, but do 1st due to access
Lab - c-ANCA, anti-PR3
Limited Wegeners - no renal involvement
Dx with bx
Rapid progression to death if untreated-cyclophos+steroids; give Bactrim due to increased risk of PCP pneumonia
Microscopic polyangiitis
pumonary-renal syndrome
necrotizing crescentric glomerulonephritis
Pauci immune (with no real immune complex deposition)
dx with bx
Leukocytoclastic vasculitis
small vesel vasculitis, esp skin
Palpable purpura, crops of purple papules, large petichae
Causes-drug rxn, viruses, SLE, RA, Henoch-Schonlein purpura, cyroglobulinemia
drug rxn - 1-10 days after starting, can be after dc'd
Henoch-Schonlein purpura
IgA mediated
Skin from waist down, kidneys (IgA nephrop), GI (abd pain, bleeding)
Usually kids, assc with recent infection
Dx - IgA on skin bx
Rx - usually resolves spontaneously, steroids or cytotoxics if severe
Goodpasture syndrome
Lung and kidney involvement
Rapidly progressive sxs - hemopysis, pulm hemorrhage, rapidly progressing glomerulonephritis
Anti-glomerular basement membrane ab positive
Pulmonary-renal syndromes
wegeners, goodpastures, microscopic polyangiitis, SLE, Churg-Strauss
C-S doesn't cause pulm hemorrhage, others do
Cryoglobulinemia
Abnormal proteins, precipitate, increase viscosity when cold
Mixed (polyclonal) - vasculitis with purpura, glomerulonephritis -assc with Hep C
Monoclonal - MM, Walenstoms
Do BCx to r/o endocarditis, which can mimic this
Cholesterol embolization
vasculitis with purpura
See in pt with severe atheroscleosis who just had angiogram
Hypereosinophilia, hypocomplementemia
dx with skin bx
Relapsing polychondritis
Inflammation of cartilidge of ear, nose, larynx, trachea; can give arthritis, ocular dz
Can cause aortic dilatation
Present with hoarseness, saddle-nose, swelling of ear cartilidge
Behcet disease
Middle east, japanese, asian decent
Multisystem - painful oral, genital ulcers, synovitis, cutaneous lesions, CNS, pulmonary art aneurysms, hyperreactive to needle sticks
Respond to colchicine, pentoxyfilline
Malignancy assc illness
hypertrophic pulmonary osteoarthropathy
Amyloidosis
gout
carcinomatous polyarthritis
Pagets dz
`hearing loss, LBP, skull enlargement
Angioid streaks on fundus
Hypervascular dz - high output CHF
Dx - elevated alk phos, urine hydroxyproline
Sarcomas in 1%
Tests for shoulder impingement syndrome
Neer test - pt shrus shoulder while you resist it, passively lift arm towards ear
Hawkins test - flex elbow, internally rotate elbow
Yocum test - touch opposite shoulder, lift up on elbow
abnormal if pain in any test
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