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NYU Medical Grand Rounds Clinical Vignette

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. Chief Complaint. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS.

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NYU Medical Grand Rounds Clinical Vignette

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  1. NYU Medical Grand Rounds Clinical Vignette Laura Van Metre Baum, MD Class of 2013 Tuesday, April 17, 2012 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

  2. Chief Complaint UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • The patient is a 23 year-old G3P0020 at 28 weeks gestation who presented with several weeks of edema, dyspnea, and arthralgias.

  3. History of Present Illness UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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

  4. History of Present Illness UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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.

  5. Additional History UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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

  6. Additional History UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • Social History: • No tobacco or drug use • Occasional alcohol use prior to pregnancy • Allergies: • NKDA • Home Medications: • Prenatal vitamins • Ferrous sulfate

  7. Physical Examination UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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.

  8. Laboratory Findings UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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 132 meq/L HCO3 15meq/L • K 5.7 meq/L BUN 65 mg/dL • Cl 105 meq/L Cr 3.2 mg/dL

  9. Laboratory Findings UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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)

  10. Laboratory Findings UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • ANA Negative • ANCA Negative • MyeloperoxidaseAb Negative • Proteinase-3 Ab Negative • Lupus anticoagulant Negative • AnticardiolipinIgG/IgM Negative • HIV 1/2 Ab Negative • HBVsAb/sAg Negative • C3 50 units (75-140) • C4 9 units (10-34)

  11. Other Studies UNITED STATES DEPARTMENT OF VETERANS AFFAIRS • 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)

  12. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Differential Diagnosis • Membranoproliferative glomerulonephritis • Lupus nephritis • ANCA vasculitis • Immune complex disease • Endocarditis • Cryoglobulinemia • Post-streptococcal glomerulonephritis

  13. 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.

  14. 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.

  15. Hospital Course (lupus nephritis preeclampsia) Gestation 28wks 29wks 30.5wks 2wkspp Day 1 5 9 22 23 IV pulse steroids azathioprine Delivery renal bx Class IV, V MMF BP 120/70 120-130/70-80 160/100 U/A no casts, +RBC, WBC U p/c 5.7 gms 5.8 gms 6.7 gms Creat 2.9 2.4 1.4 1.1 U.A. 10.1 11.0 6.1 Abs DNA >300 113 69 C3 44 57 91 C4 9 9 15 No IUGR sFlt-1 = 9,471sFlt-1 = 15,038 pg/ml

  16. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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)

  17. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Final Diagnosis • ANA negative lupus nephritis complicated by pre-eclampsia

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