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WEGENER’S GRANULOMATOSIS

WEGENER’S GRANULOMATOSIS. Definition. A multisystem disease characterized by granulomatous vasculitis involving multiple organs. upper airway (otitis, sinusitis, nasal mucosa) lung kidney Other organ systems involved include skin, joints, nervous system (peripheral or central).

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WEGENER’S GRANULOMATOSIS

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  1. WEGENER’S GRANULOMATOSIS

  2. Definition • A multisystem disease characterized by granulomatous vasculitis involving multiple organs. • upper airway (otitis, sinusitis, nasal mucosa) • lung • kidney Other organ systems involved include skin, joints, nervous system (peripheral or central).

  3. EPIDEMIOLOGY • Incidence/Prevalence in USA: Incidence estimated at approximately 0.4/100,000; prevalence 3/100,000 • Predominant age: Mean age of onset in mid-40's, but has been described in all age groups • Predominant sex: Male > Female (3:2)

  4. Which one is seen in WG?

  5. Which one is nasal crust?

  6. SIGNS & SYMPTOMS • Pulmonary infiltrates (71%) • Sinusitis (67%) • Arthralgia/arthritis (44%) • Fever (34%) • Cough (34%) • Otitis (25%) • Rhinitis (22%)

  7. SIGNS & SYMPTOMS • Hemoptysis (18%) • Ocular inflammation (16%) • Weight loss (16%) • Skin rash (13%) • Epistaxis (11%) • Renal failure (11%) • Chest pain, anorexia, proptosis, dyspnea, oral ulcers, hearing loss, headache (all < 10%)

  8. DIFFERENTIAL • Infectious otitis and sinusitis (bacterial or fungal) • Midline granuloma or other upper airway malignancy • Fungal or tuberculous pulmonary infections, (Goodpasture's syndrome)

  9. DIFFERENTIAL • Other vasculitic syndromes (including polyarteritis nodosa, lymphomatoid granulomatosis, Churg-Strauss vasculitis, and overlap vasculitis syndromes) • Any disease associated with necrotizing and crescentic glomerulonephritis

  10. LABS • Anemia, leukocytosis, and thrombocytosis common during active phases of disease • Erythrocyte sedimentation rate (ESR) usually markedly elevated (75%) • Rheumatoid factor present in low to moderate titer in up to 50%

  11. LABS • Hematuria and/or cellular casts with moderate range proteinuria • Renal insufficiency, mild to moderate at first, but frequently progresses to end-stage renal disease

  12. SPECIAL TESTS • Antibodies to neutrophilic cytoplasmic antigens with a cytoplasmic pattern of staining (c-ANCA) are detected in a majority (60-90%) of patients. Such pattern of staining is highly specific (90+%) for this diagnosis.

  13. Special Tests • Perinuclear staining (p-ANCA), is nonspecific, but frequently seen in patients with other vasculitic syndromes or isolated necrotizing glomerulonephritis.

  14. TREATMENT • Prednisone - given initially in high doses (60-100 mg/day). After initial 2-4 weeks may be tapered to alternate-day regimen. Then gradually discontinued over 2-6 months in most patients, depending on clinical course.

  15. TREATMENT • Cyclophosphamide - in critically ill patient, may be given initially at a dose of 4 mg/kg/day IV for 2-3 days, then continued at 2 mg/kg/day orally. In stable patient, may be started at 2 mg/kg/day orally. Dosage may need to be adjusted, based on patient response and toxicity (usually bone marrow suppression).

  16. Complications • Disease related • Destructive nasal lesions with "saddle nose" deformity • Deafness from refractory otitis • Necrotic pulmonary nodules with hemoptysis • Interstitial lung disease

  17. Complications • Renal failure • Foot drop from peripheral nerve disease • Skin ulcers, digital and limb gangrene from peripheral vascular involvement

  18. Prognosis • Without treatment, almost uniformly fatal with 10% 2 year survival and mean survival of 5 months • With aggressive treatment, survival improved to 75-90% at 5 years • Treatment-related toxicity is significant, especially from long-term cyclophosphamide. After 1-2 years of disease-free interval, cyclophosphamide is usually tapered, although some patients demonstrate disease re-activation during this phase.

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