resident report
DESCRIPTION
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 - PowerPoint PPT PresentationTRANSCRIPT
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Resident Report 8.25.11
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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
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Types of Vasculitis
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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
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Sequelae
• Nasal septal perforation• Airway stenosis• Renal failure
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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%
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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
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Treatment
• Cyclophosphamide (alkylating agent) and steroids • Induce remission in most patients (> 80%)
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Alternative Regimens
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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
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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