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Granulomatosis with polyangiitis
From WikEM
(Redirected from Wegener's granulomatosis)
Contents
Background
- Formerly known as Wegener's granulomatosis
- c-ANCA associated systemic necrotizing vasculitis
- Small- and medium-sized blood vessels
- Predilection for upper and lower respiratory tracts and kidneys
Clinical Features
- Upper respiratory, pulmonary and renal disease + constitutional symptoms
- White, older patients
- Constitutional symptoms: Fever, malaise, weight loss
- Upper airway: Serous otitis media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease
- Subglottic stenosis is most common laryngotracheal lesion (16% patients)
- Lower respiratory: Cough, dyspnea, pleuritis, hemoptysis, diffuse alveolar hemorrhage
- Renal failure, glomerulonephritis
- Ophthalmologic: scleritis, episcleritis, uveitis
- Cutaneous: Palpable purpura, nodules, ulcers
- Neurologic: Mononeuropathy and polyneuropathy, cerebral vasculitis, cerebral hemorrhage or thrombosis
- Cardiac: Pericarditis, myocarditis
Differential Diagnosis
- Polyarteritis nodosa, Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), SLE, Goodpasture syndrome
- Lymphoma, lung cancer
- Pneumonia, infective endocarditis, HUS
- Glomerulonephritis
Evaluation
Classification
- American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria
- Nasal or oral inflammation
- Abnormal chest radiograph showing nodules, fixed infiltrate, or cavities
- Abnormal urinary sedimentation (microscopic hematuria)
- Granulomatous inflammation on biopsy of an artery or perivascular area
Workup
- Definitive diagnosis: Biopsy
- ANCA +, RF+
- CBC: Leukocytosis, normochromic anemia, thrombocytosis
- ESR/CRP elevated
- BUN/Cr
- Urinalysis (hematuria, proteinuria)
- CXR- Pulmonary infiltrates and nodules
- CT chest
- To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood culture to rule out other pathology
- Other tests: Bronchoscopy, PFT, sinus CT
Management
- Priority: Manage pulmonary hemorrhage and renal insufficiency
- Difficult airway: Diffuse alveolar hemorrhage and subglottic stenosis
- Fiberoptic intubation through LMA advocated
- Difficult airway: Diffuse alveolar hemorrhage and subglottic stenosis
- Rheumatology consult + multidisciplinary consults
- Mild disease: Corticosteroids and methotrexate
- No active glomerulonephritis or organ-threatening disease
- Mod-Severe disease: Corticosteroids and cyclophosphamide or rituximab
- Corticosteroids:
- Methylprednisolone (7-15mg/kg/d with max 1000mg)
- Prednisone 1mg/kg/d (max 80mg)
- Methotrexate: 20-25mg weekly PO or SC
- Cyclophosphamide: 2mg/kg/d PO or 15mg/kg q2 weeks x3 then q3 weeks
- Rituximab: 357mg/m² weekly x 4
- To consider plasma exchange: Severe/rapidly progressive renal disease, concurrent anti-GBM Ab disease, severe pulmonary hemorrhage
