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NYU Medical Grand Rounds Clinical Vignette Laura Van Metre Baum, MD Class of 2013 Tuesday, April 17, 2012 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Chief Complaint •The patient is a 23 year-old G3P0020 at 28 weeks gestation who presented with several weeks of edema, dyspnea, and arthralgias. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS History of Present Illness •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 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS History of Present Illness •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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Additional History •Social History: •No tobacco or drug use •Occasional alcohol use prior to pregnancy •Allergies: •NKDA •Home Medications: •Prenatal vitamins •Ferrous sulfate UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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/L HCO3 15meq/L •K 5.7 meq/L BUN 65 mg/dL •Cl 105 meq/L Cr 3.2 mg/dL UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Laboratory Findings •Urinalysis •3+ protein, 3+ blood, 3+ leuk esterase •WBC 30-50 •RBC 5-10 •24h urine protein 5.7g •sFlt-1 9471 pg/mL (<4500pg/mL) (soluble fms-like tyrosine kinase-1, a predictor of severe pre-eclampsia) UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Laboratory Findings •ANA •ANCA •Myeloperoxidase Ab •Proteinase-3 Ab •Lupus anticoagulant •Anticardiolipin IgG/IgM Negative Negative Negative Negative Negative Negative •HIV 1/2 Ab •HBVsAb/sAg Negative Negative •C3 •C4 50 units (75-140) 9 units (10-34) UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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) UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Differential Diagnosis • • • • • • • Membranoproliferative glomerulonephritis Lupus nephritis ANCA vasculitis Immune complex disease Endocarditis Cryoglobulinemia Post-streptococcal glomerulonephritis UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Hospital Course • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Hospital Course • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Hospital Course (lupus nephritis Gestation 28wks Day 1 29wks 9 5 IV pulse steroids BP 120/70 U/A no casts, +RBC, WBC U p/c 5.7 gms Creat 2.9 U.A. 10.1 Abs DNA >300 C3 44 C4 9 preeclampsia) 30.5wks 2wkspp 22 23 azathioprine Delivery 120-130/70-80 160/100 5.8 gms 2.4 11.0 113 57 9 6.7 gms 1.4 6.1 69 91 15 No IUGR sFlt-1 = 9,471 sFlt-1 = 15,038 pg/ml renal bx Class IV, V MMF 1.1 Hospital Course • 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) UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Final Diagnosis • ANA negative lupus nephritis complicated by pre-eclampsia UNITED STATES DEPARTMENT OF VETERANS AFFAIRS