![logo]()
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 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 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 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 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 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 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) 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 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) 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 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 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 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" 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 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. 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 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 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 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 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 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 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 