1. NYU Medical Grand Rounds Clinical Vignette Laura Van Metre Baum, MD Class of 2013 Tuesday, April 17, 2012 U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

2. The patient is a 23 year-old G3P0020 at 28 weeks gestation who presented with several weeks of edema, dyspnea, and arthralgias. Chief Complaint U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

3. She was in good health with a normal pre-natal course until approximately 10 weeks gestation when she was noted to have proteinuria and acute kidney injury. Over the subsequent 18 weeks, while followed in an outpatient obstetrics clinic, she developed: Worsening proteinuria Rising creatinine (1.0 to 2.9mg/dL) Progressive dyspnea on exertion Swelling and pain in multiple fingers as well as her R wrist and elbow Lower extremity edema progressing to anasarca History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

4. She initially presented to Queens Hospital where she was empirically treated with intravenous corticosteroids. She went on to develop oliguric acute on chronic kidney injury before being transferred to Bellevue Hospital for further care. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

5. Additional History Past Medical History: Two spontaneous first-trimester abortions Childhood asthma Iron deficiency anemia Past Surgical History: D&C for intrauterine fetal demise Family History: Father – end-stage renal diseases due to hypertension – s/p renal transplant Mother: pre-eclampsia U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

6. Additional History Social History: No tobacco or drug use Occasional alcohol use prior to pregnancy Allergies: NKDA Home Medications: Prenatal vitamins Ferrous sulfate U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

7. Physical Examination Grossly anasarcic, gravid woman in no acute distress. Vital Signs: T 98.0F, HR 70, BP120/70 RR16, SaO2 100%Room Air 2+ pitting edema in upper and lower extremities. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

8. Laboratory Findings CBC: WBC 21.8 (N 92%, L 4%, Mono 4%) Hemoglobin 9.6mg/dl (s/p 2units PRBC at Queen’s Hospital) Platelets 246,000 Basic Metabolic Na 132meq/LHCO3 15meq/L K 5.7 meq/LBUN 65 mg/dL Cl 105meq/LCr 3.2 mg/dL U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

9. Laboratory Findings Urinalysis 3+ protein, 3+ blood, 3+ leuk esterase WBC 30-50 RBC 5-10 24h urine protein5.7g sFlt-19471 pg/mL (<4500pg/mL) (soluble fms-like tyrosine kinase-1, a predictor of severe pre-eclampsia) U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

10. Laboratory Findings ANANegative ANCANegative Myeloperoxidase AbNegative Proteinase-3 AbNegative Lupus anticoagulantNegative Anticardiolipin IgG/IgMNegative HIV 1/2 AbNegative HBVsAb/sAgNegative C350 units (75-140) C49 units (10-34) U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

11. Other Studies Renal Ultrasound R Kidney10.4cm; L kidney 11.8cm Increased echogenicity CXR: normal TTE: Normal LVEF Moderate mitral and tricuspid insufficiency Mild pulmonary insufficiency Moderate pulmonary hypertension (PASP 52mmHg) U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

12. Membranoproliferative glomerulonephritis Lupus nephritis ANCA vasculitis Immune complex disease Endocarditis Cryoglobulinemia Post-streptococcal glomerulonephritis Differential Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

13. She was started on pulse dose methylprednisolone followed by maintenance therapy. Several days later, her dsDNA results were positive with titer >300 units (>=10 is positive). Her renal function worsened despite high dose steroids. Azathioprine was added. Cyclophosphamide was avoided due to teratogenicity and fetal harm. Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

14. Necrotizing vulvar infection requiring debridement and washout. She developed hypertension and pre-eclampsia. C-section was performed at 30+5 weeks. A 1.3kg male infant was delivered –APGAR 6 and 8. Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

15. Hospital Course (lupus nephritis preeclampsia) Gestation 28wks 29wks 30.5wks 2wkspp BP 120/70120-130/70-80 160/100 U/A no casts, +RBC, WBC U p/c 5.7 gms5.8 gms 6.7 gms Creat 2.92.4 1.41.1 U.A. 10.1 11.0 6.1 Abs DNA >300113 69 C3 4457 91 C4 9 9 15 sFlt-1 = 9,471sFlt-1 = 15,038 pg/ml No IUGR IV pulse steroids azathioprine Delivery renal bx Class IV, V MMF Day 1 5 9 22 23

16. After delivery, a renal biopsy was performed: Pathology Results –Segmental diffuse proliferative glomeruloneprhitis –Focal cellular and fibrocellular crescents –Membranous glomerulopathy consistent with lupus nephritis (Class IV & V) Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

17. ANA negative lupus nephritis complicated by pre-eclampsia Final Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS