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Overview of Small Vessel Vasculitis: Symptoms, Diagnosis, and Treatment Options

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matana
11 diapositives · 253 Views · Jul 19, 2014

Small vessel vasculitis, including Wegener's granulomatosis, presents with severe symptoms affecting the sinus, upper airway, lungs, and kidneys. Without treatment, it can lead to death within weeks to months. Key symptoms include malaise, hematuria, and pulmonary issues such as cough and hemoptysis. Diagnosis requires fulfilling specific ACR criteria and testing for ANCA antibodies. Treatment options include cyclophosphamide and rituximab, with the latter offering a favorable safety profile for younger patients. Understanding this condition is essential for effective management.

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  1. Resident Report 8.25.11

  2. 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

  3. Types of Vasculitis

  4. 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

  5. Nasal septal perforation • Airway stenosis • Renal failure Sequelae

  6. 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

  7. 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

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

  9. Alternative Regimens

  10. 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

  11. 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