NYU Medicine Grand Rounds Clinical Vignette James Kim, M.D., PGY-2 February 26, 2014 U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

53 year-old woman presents with pain in multiple joints (knees, wrists, shoulders, elbows, ankles) for 2 months and fatigue, shortness of breath and hemoptysis for 1 week Chief Complaint U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

1 year prior to current presentation, presented with left sided vision changes – pink spots alternating with darkness. Found to have elevated ESR/CRP and prescribed course of prednisone for optic neuritis Underwent a temporal artery biopsy, which was non-diagnostic, and the patient’s vision returned to normal after completing prednisone course 2 months ago, began to develop fluctuating pain in multiple joints accompanied by swelling, mildly relieved by ibuprofen 1 week ago, began to develop generalized fatigue, shortness of breath, and blood-streaked sputum Due to worsening symptoms, she presented to the hospital History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Additional History Past Medical History: Left optic neuritis Seasonal allergies Past Surgical History: None Social History: No tobacco, alcohol, illicit drugs Family History: No notable family history Allergies: Acetaminophen (rash) Medications: Ibuprofen as needed U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Physical Examination General: Pale, no acute distress Vital Signs: T: 97.8 F BP: 141/81 HR: 102 RR: 18 and O2 sat: 100% 2L nasal cannula Conjunctival pallor Heberden nodes on all digits Trace right knee effusion without warmth, erythema, tenderness Remainder of physical exam was normal U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Laboratory Findings CBC: hemoglobin 4.6 g/dL, hematocrit 13.9% Remainder of CBC was within normal limits Basic Metabolic panel: within normal limits Hepatic panel: within normal limits ESR: 60 mm/hr (0-20) CRP: 69.9 mg/L (<= 3.0) C-ANCA: < 6 (<1.0) P-ANCA: >100 (<1.0) Urinalysis: 3+ blood, RBC 15-30/hpf (0-4/hpf) U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Other Studies Chest X-Ray: diffuse tiny nodular infiltrates scattered throughout both lungs Chest CT with contrast: ground glass density in the mid lung fields that becomes more confluent at the bases. Diffuse subcentimeter nodules. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Microscopic Polyangiitis Polyarteritis Nodosa Churg-Strauss Syndrome Working or Differential Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Hospital Day 1: –Started on IV methylprednisolone, and transfused 2 units of packed red blood cells –Diffuse alveolar hemorrhage seen on bronchoscopy. Transbronchial biopsy showing no evidence of vasculitis Hospital Day 2: –Hematocrit and symptoms improved following transfusion and steroids Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Hospital Course Hospital Day 3: -Hematocrit remained stable, methylprednisolone changed to daily prednisone -Renal biopsy obtained, consistent with ANCA associated, pauci-immune crescentic glomerulonephritis -Hospital Day 4: -Discharged with close follow up U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Microscopic Polyangiitis Final Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS