Granulomatosis with polyangiitis

Background

• "Granulomatosis with polyangiitis"
• 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
• Constitutional symptoms: Fever, malaise, weight loss
• Upper airway: Serous otits media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease
  • Subglottic stenosis MC 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

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
• CBC: Leukocytosis, normochronic anemia, thrombocytosis
• ESR/CRP elevated
• BUN/Cr
• UA (hematuria, proteinuria)
• CXR- Pulmonary infiltrates and nodules
• CT chest
• To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood cx to r/o other pathology

Management

• Priority: Managing pulmonary hemorrhage and renal insufficiency
• Difficult airway: Diffuse alveolar hemorrhage and subglottic stenosis
  • Fiberoptic intubation through LMA advocated
• Corticosteroids and cyclophosphamide

Sources

• Reference: Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p.