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Resident Report . 8.25.11. Small vessel vasculitis Typical areas affected are sinus, upper airway, lungs, kidney Progressive course with death in weeks to months if no treatment. Wegener’s Granulomatosis. Types of Vasculitis. Constitutional – malaise, fatigue, weight loss
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Resident Report 8.25.11
Small vessel vasculitis • Typical areas affected are sinus, upper airway, lungs, kidney • Progressive course with death in weeks to months if no treatment Wegener’s Granulomatosis
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 Clinical Symptoms
Nasal septal perforation • Airway stenosis • Renal failure Sequelae
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% Diagnosis
Testing • ANCA – typically see elevated c-ANCA (> 80% positive) • Anti-proteinase 3 (anti-PR3) is specific • Biopsy of affected tissues shows necrotizing granulomatous vasculitis ANCA-associated granulomatous vasculitis
Cyclophosphamide (alkylating agent) and steroids • Induce remission in most patients (> 80%) Treatment
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 RAVE TRIAL
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 Naming Controversy