1. NYU Medical Grand Rounds Clinical Vignette Matko Kalac, MD PGY-2 9/18/2012 U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

2. The patient is a 35-year-old Hispanic female with six years of dark urine and urinary urgency. Chief Complaint U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

3. The patient presented to clinic with intermittently brownish urine and urinary urgency for six years. Over the past year she developed an erythematous pruritic rash over her arms, legs, face, chest and abdomen. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

4. She also reported swelling of her fingers and lower extremities bilaterally, associated with pain and morning stiffness in her fingers that last several minutes. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

5. Additional History Past Medical History: Pyelonephritis Gestational diabetes Past Surgical History: None Social History: No smoking, no alcohol or illicit drug use Family History: No history of kidney disease or arthritis Allergies: No known drug allergies Medications: Ibuprofen as needed U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

6. Physical Examination General: Patient was in no apparent distress Vital Signs: T: 97.1 BP:118/68 HR:77 RR:18 O2 saturation: 98% Systolic murmur 2/6 best heard over left lower sternal border, 1+ bilateral lower extremity edema Purpura on lower extremities, abdomen and chest, diffuse scattered erythematous papular eruptions on upper extremities Remainder of physical exam was normal U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

7. Laboratory Findings Complete Blood Count was within normal limits Basic Metabolic panel was within normal limits Hepatic Panel: Albumin 3.3 g/dL Lipid Panel: - Cholesterol 194 mg/dL - Triglycerides 378 mg/dL - HDL 52 mg/dL - LDL 66 mg/dL U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

8. Further Laboratory Findings Urinalysis: - Specific gravity 1.012, - Protein > 300mg/dL, - Positive nitrite - WBC 5-10 - RBC 15-30 - Many bacteria - Coarse granular casts 2-5 Urine random protein 742mg/dL Random creatinine 106.7 mg/dL Protein/creatinine ratio 7 24h urine protein 8.9g U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

9. Laboratory Findings ANA, anti-dsDNA, rapid HIV negative Hepatitis B and C negative Complement levels were normal A skin biopsy showed urticarial vasculitis. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

10. Differential diagnosis – nephritic syndrome with nephrotic range proteinuria: –Henoch-Schonlein purpura –Subacute ANCA associated vasculitis –Mixed cryoglobulinemic syndrome –Early ANA negative lupus with membranous and some proliferative features Differential Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

11. -Kidney biopsy showed necrotizing and cresentic glomerulonephritis with mesangial IgA and segmental sclerosis consistent with IgA nephropathy -Of 27 and 29 glomeruli on biopsy, 2 were globally sclerotic and an additional 3/27 and 12/29 were segmentally sclerotic -The patient was started on lisinopril and prednisone 1mg/kg daily Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

12. Henoch-Schoenlein purpura with crescentic IgA nephropathy Final Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS