1. Resident Report 8.25.11

2. 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

3. Types of Vasculitis

4. 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

5. Sequelae Nasal septal perforation Airway stenosis Renal failure

6. 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%

7. 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

8. Treatment Cyclophosphamide (alkylating agent) and steroids Induce remission in most patients (> 80%)

9. Alternative Regimens

10. 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

11. Naming Controversy Dr. Wegener was a member of the Nazi party No proof that he engaged in war-crimes, but given his involvement unthinkable he was oblivious to holocaust 1989 given ACP Master Clinician Award 1 year later, his Nazi past was publicly known and award eventually revoked