Resident Report 8.25.11. Wegener’s Granulomatosis Small vessel vasculitis Typical areas affected are sinus, upper airway, lungs, kidney Progressive course.

Resident Report 8.25.11

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

Types of Vasculitis

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

Sequelae

• Nasal septal perforation• Airway stenosis• Renal failure

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%)

Alternative Regimens

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

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