WEGENER’S GRANULOMATOSIS
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Transcript of WEGENER’S GRANULOMATOSIS
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WEGENERS GRANULOMATOSIS
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DefinitionA 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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EPIDEMIOLOGYIncidence/Prevalence in USA: Incidence estimated at approximately 0.4/100,000; prevalence 3/100,000Predominant age: Mean age of onset in mid-40's, but has been described in all age groupsPredominant sex: Male > Female (3:2)
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Which one is seen in WG?
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Which one is nasal crust?
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SIGNS & SYMPTOMSPulmonary infiltrates (71%) Sinusitis (67%) Arthralgia/arthritis (44%) Fever (34%) Cough (34%) Otitis (25%) Rhinitis (22%)
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SIGNS & SYMPTOMSHemoptysis (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%)
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DIFFERENTIALInfectious otitis and sinusitis (bacterial or fungal) Midline granuloma or other upper airway malignancy Fungal or tuberculous pulmonary infections, (Goodpasture's syndrome)
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DIFFERENTIALOther vasculitic syndromes (including polyarteritis nodosa, lymphomatoid granulomatosis, Churg-Strauss vasculitis, and overlap vasculitis syndromes) Any disease associated with necrotizing and crescentic glomerulonephritis
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LABSAnemia, 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%
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LABSHematuria and/or cellular casts with moderate range proteinuria Renal insufficiency, mild to moderate at first, but frequently progresses to end-stage renal disease
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SPECIAL TESTSAntibodies 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.
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Special TestsPerinuclear staining (p-ANCA), is nonspecific, but frequently seen in patients with other vasculitic syndromes or isolated necrotizing glomerulonephritis.
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TREATMENTPrednisone - 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.
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TREATMENTCyclophosphamide - 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).
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ComplicationsDisease related Destructive nasal lesions with "saddle nose" deformity Deafness from refractory otitis Necrotic pulmonary nodules with hemoptysis Interstitial lung disease
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ComplicationsRenal failure Foot drop from peripheral nerve disease Skin ulcers, digital and limb gangrene from peripheral vascular involvement
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PrognosisWithout 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.