Post on 19-Dec-2015
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Wegener’s Granulomatosis
• Small vessel vasculitis• Typical areas affected are sinus, upper airway, lungs,
kidney• Progressive course with death in weeks to months if no
treatment
Clinical Symptoms
• Constitutional – malaise, fatigue, weight loss• Renal – hematuria, oliguria• Pulm – cough, hemoptysis, pleuritic pain• HEENT – sinusitis, otitis• MSK – arthralgias, myalgias• Neuro – peripheral neuropathy, mononeuritis• Ocular – Conjunctivitis, scleritis
Diagnosis
• ACR Criteria• Sinus involvement• Pulmonary abnormality on CXR• Abnormal Urinary Sediment• Biopsy of affected tissue showing granulomatous
inflammation
• If 2/4 positive, sensitivity is 88% and specificity is 92%
ANCA-associated granulomatous vasculitis
• Testing• ANCA – typically see elevated c-ANCA (> 80% positive)• Anti-proteinase 3 (anti-PR3) is specific• Biopsy of affected tissues shows necrotizing granulomatous
vasculitis
Treatment
• Cyclophosphamide (alkylating agent) and steroids • Induce remission in most patients (> 80%)
RAVE TRIAL
• Rituximab is a B cell inhibitor that does not have the same side effects as cyclophosphamide• Bone marrow suppression, elevated infection risk,
infertility, hemorrhagic cystitis• Makes it more usable, especially in younger patients
• Trial showed non-inferiority of rituximab as compared to cyclophosphamide in inducing remission of ANCA-associated vasculitis