- Classic Triad (lung, kidney, skin)
- Upper and lower respiratory tracts
- Glomerulonephritis
- Skin including oral mucosa
- Necrotizing granulomatous inflammation
- c-ANCA (80%) > p-ANCA (15%) - correlate with disease activity
Apresentação clínica
- Nasal, sinus, tracheal or ear involvement
- 73% at presentation (rhinorrhea, sinusisits, ulceration)
- Can result in Saddle Nose deformity (from septal perforation)
- CXR with infiltrates or effusions (later in disease)
- Pauciimmune crescentic glomerulonephritis (pyuria, proteinuria, hematuria)
- Fever, weight loss, malaise, cough, dyspnea
- 20% present with skin involved; 40% will develop
- Nodules, papules, vesicles, petechiae, retiform purpura, pyoderma gangrenosum-like lesions
Treatment/prognosis
- Initial: cyclophosphamide 2mg/kg/day and prednisone 1mg/kg/day
- Maintenance: MTX, azathiprine, mycophenolato mofetil
- Untreated: mean survival 5 months
- Remission in 93% for 4 years in those treated, however high remission rates if discontinued
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