Transcript Journal Club

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