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- Microscopic Polyangiitis (MPA)
- Course
- Affected : nose , throat , bronchi , kidneys Complications
- Associations
- Chest CT
- Hypertrophic pachymeningitis (HP)
- Raynaud’s
- Asthma
- Hypersensitivity Angiitis
- Joints
- Less common
- Arthritis
Labs: elevated ESR (~85), CRP, WBC, eosinophils, normochromic anemia, HBsAg found in 10-30%, HCV rarely associated, 20% have positive p-ANCA to myeloperoxidase (MPO), RF (+) in 20% Treatment: steroids, cyclophosphamide, plasma exchange (2nd line) HBV-PAN More HTN than classic PAN Remember to treat both HBV and PAN Treatment: plasmapheresis and lamivudine Microscopic Polyangiitis (MPA) May have capillary involvement in addition to small/medium arteries (unlike classic PAN) Glomerulonephritis (usu. RPGN) common (not in PAN), alveolar hemorrhage (not in PAN)
(longer prodrome than with PAN) / most with constitutional symptoms before diagnosis Labs: almost always have p-ANCA (+) and MPO (+) / rarely c-ANCA will be (+) / other P-ANCA (but not MPO): Felty’s, UC (directed against different proteins) Course: relapse in MPA (35%) > HBV-PAN and c-PAN (10%) Treatment: ?similar to classic PAN Wegener’s Granulomatosis medium to small arteries / type IV DTH Presentation: chronic sinusitis, epistaxis, mucosal ulcers, cough (45%), hemoptysis (30%), fever, night sweats, arthritis, myalgia, skin nodules, renal failure, cranial nerve palsies (II, VI, VII), pachymengitis / mean interval from symptoms to diagnosis 15 months Affected: nose, throat, bronchi, kidneys Complications: renal failure (hematuria, RBC casts, RPGN) / saddle-nose deformities, sepsis, hemorrhage, DIC / usu. does not lead to respiratory failure Associations: Hodgkin’s lymphoma Diagnosis: cANCA (confirm with anti-proteinase 3) (90% sensitivity, few false positives) / ~10% with p-ANCA / ~10% with anti-GBM biopsy of ENT likely to show only necrosis, biopsy of kidney likely to show only non-specific RPGN, you can’t stain for IF (that’s why it’s called pauci-immune!!!), biopsy of lung most likely to confirm diagnosis Chest CT [CT] [CT] [CT] pulmonary infiltrates or nodules (up to 85%) / not pleural effusions Labs: elevated ESR, thrombocytosis Prognosis: higher ESR, older age gives worse prognosis / ENT involvement gives better prognosis Treatment: cyclophosphamide (1st), steroids (2nd or 1st for pulmonary hemorrhage) / bactrim is sometimes helpful (prevention of infection may avoid a potential trigger) / not MTX Hypertrophic pachymeningitis (HP) pANCA positive CNS disease, which some think of as Wegener’s limited to CNS/cranial nerves / treat as Wegener’s
male, smokers / medium to small arteries AND veins Presentation: hypercoagulability, claudication, pain, Raynaud’s, gangrene Exam: Allen’s test Treatment: perhaps ca-blockers, stopping smoking will hopefully stop progression of disease
3 stages (order can vary) / asthma, eosinophilia, vasculitis Asthma may occur up to 30 yrs (mean 3) before vasculitis, onset with vasculitis in 10%, after in 2%) / sinusitis, allergic rhinitis, nasal polyps Eosinophilia can mimic chronic eosinophilic pneumonia Vasculitis mononeuritis multiplex (72%), weight loss (>50%), fever, myalgia, skin lesions (60%) > GI (50%) > spleen > heart (myocarditis, infarction) > kidneys (FSGN) Diagnosis: angiitis and extravascular necrotizing granulomas with eosinophils Labs: P-ANCA (50%, usu. anti-MPO), RF, elevated ESR (80% of cases), eosinophilia (over 10% in 90% of cases) Often seen in asthma patients being tapered from PO or inhaled steroids (may be associated with leukotriene antagonists) Treatment:
adverse drug reaction HSP (Henoch Schönlein Purpura) (see renal) small vessel vasculitis Behçet’s Disease (Behcets) [NEJM] mostly in people of Middle East, Japanese descent / onset in 20 to 30s Course: chronic, relapsing acute attacks / manifestations (except uveitis) usually self-limited Presentation: Mouth: aphthous oral [pic] / genital ulcers [pic] Eye: uveitis, retinitis (can cause blindness), hypopyon Skin: superficial migratory thrombophlebitis, erythema nodosum (including pseudofolliculitis and acneiform nodules / pathergy Joints: mono/polyarthritis (50%, knees > wrist, elbows, ankles) (non-deforming) Coagulopathy: vasculitis/ hypercoagulability (major concern is retino-occlusive disease), venous 7 times more than arterial (however, can get aneurysms, stenoses), 25% will have at least superficial venous thromboembolism Less common: GI, CNS (early onset males 10-20%), large vessels / may have Diagnosis: oral ulcers + 2 other criteria / can look for HLA-B51 (not in S. American, N. American), elevated IgD levels CSF: elevated IgG (not oligoclonal), pleocytosis MRI: multiple high-intensity focal lesions in brain stem, basal ganglia, and cerebral white matter are typical on T2-weighted MRI Ddx: chronic oral aphthosis, Sweet’s, HSV, AS / GI (IBD), CNS (MS), pathergy (Sweet’s, pyoderma gangrenosum), retinitis (sarcoid, viral retinitis) Treatment: Skin: topical steroids, thalidomide, colchicines, oral steroids Ocular, GI, CNS: oral steroids / other immunosuppressives (Cytoxan, Immuran, others) / note: cyclosporin may worsen CNS symptoms (it’s not a first line agent) / IFN-a, IVIG under investigation Arthritis: steroids, NSAIDS, colchicines, sulfasalazine, IFN-a (highly effective) Vasculitis: steroids / be careful with anticoagulation for venous thrombosis (can get big time hemoptysis with arteritis) / treatment of vascular complications is very tricky in this disease since mechanisms are multiple and unclear / CV surgery for complications HLA Associations [HLA genetics diagram]
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