NYU Medical Grand Rounds Clinical Vignette

Laura Van Metre Baum, MD

Class of 2013

Tuesday, April 17, 2012

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.

Chief Complaint

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

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.

History of Present Illness

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 132 meq/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 tyrosinekinase-1, a predictor ofsevere pre-eclampsia)

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS

Laboratory Findings

•ANA Negative•ANCA Negative•Myeloperoxidase Ab Negative•Proteinase-3 Ab Negative•Lupus anticoagulant Negative•Anticardiolipin IgG/IgM Negative

•HIV 1/2 Ab Negative•HBVsAb/sAg Negative

•C3 50 units (75-140) •C4 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

• Membranoproliferative glomerulonephritis

• Lupus nephritis

• ANCA vasculitis

• Immune complex disease

• Endocarditis

• Cryoglobulinemia

• Post-streptococcal glomerulonephritis

Differential Diagnosis

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS

• 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

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS

• 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

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS

Hospital Course (lupus nephritis preeclampsia)

Gestation28wks 29wks 30.5wks 2wkspp

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

sFlt-1 = 9,471 sFlt-1 = 15,038 pg/ml

No IUGR

IV pulse steroids azathioprine Delivery renal bx

ClassIV, VMMF

Day 1 5 9 22 23

• 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

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS

• ANA negative lupus nephritis complicated by pre-eclampsia

Final Diagnosis

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS