Sarah Williams: The Case

HPI:

Sarah Williams is a 54-year-old Caucasian female who is being seen at her primary care’s office for worsening swelling in her lower extremities. She has noticed some degree of swelling in her legs for the past couple months but feels like it has been considerably worse recently. She notes that this has been associated with unintentional weight gain and worsening fatigue. She denies chest pain, shortness of breath, nausea, vomiting, diarrhea, abdominal pain or skin changes. She had been started on Furosemide 40 mg daily during her last appointment to help with the swelling but notes her symptoms have not improved. 

Upon reviewing her chart, you note the following history:
•    Medical history: high blood pressure (on Lisinopril), nephrolithiasis (once in her 30’s, passed without intervention)
•    Surgical history: Hysterectomy (age 48)
•    Family history: Mother had congestive heart failure, high blood pressure and diabetes. Father had prostate cancer. Younger sister is healthy.
•    Social history: Current smoker (20 pack year history), drinks alcohol socially, former marijuana and cocaine use, lives at home with husband, does not work outside of the home, has no children

Vitals in the office today:

Stat	Value
HR	88
BP	152/78
T	97.8 F
RR	18
SpO2	98% on RA

On physical exam, the patient has notable 3+ pitting edema in her lower legs and 1+ pitting edema to her bilateral arms. Additionally, her eyelids appear mildly swollen. She also has some bruising on her right arm which she notes was from a recent fall.

A urinalysis is obtained and it is noted that the patient’s urine appears foamy. Labs and a chest x-ray are ordered. She is instructed to continue taking the furosemide for now. The patient informs her primary care that she relies on the bus for transportation since she cannot drive and that it might be a while before she’s able to get blood work and imaging done. When asked if someone else could bring her, she looks away and states that she doesn’t want to bother her husband. She notes losing contact with her extended family “years ago” and insists that she’ll have the blood work done when she can.
 

Abnormal urinalysis results come back several days later. The patient’s primary care attempts to contact her but the number provided goes straight to voice mail. Her provider leaves a brief message for her to call the office back. Several months later, the primary care gets more results back. The patient’s chest x-ray is shown below.

Labs are as follows:

CBC

Stat	Value
WBC	9 x 103/ul
RBC	3.2 x 106/ul
HGB	10.1 gm/dL
MCV	80 fL
Platelets	439 x 103/ul

CMP

Stat	Value
Sodium	142 mEq/L
Potassium	3.2 mEq/L
Chloride	101 mEq/L
Bicarbonate	23
Calcium	9.1 mg/dL
Protein	3.8 gm/dL
Albumin	1.7 U/L
Glucose	127 mg/dL
BUN	23 mg/dL
Creatinine	1.5 mg/dL
Bilirubin total	0.9 mg/dL
Alkaline phosphatase	97 U/L
AST	31 U/L
ALT	44 U/L

Urinalysis

Stat	Value
Urine type	Clean catch
Urine color	Yellow
Urine appearance	Foamy
Urine specific gravity	1.02
Urine pH Dipstick	7
Urine Leukocyte Esterase	Negative
Urine Nitrite	Negative
Urine protein dipstick	4+
Urine glucose dipstick	Trace
Urine ketones dipstick	Negative
Urine urobilinogen dipstick	0.7 U/dL
Urine bilirubin dipstick	Negative
Urine blood dipstick	1+
WBC	0-2/HPF
RBC	10/HPF
Bacteria	Trace
Casts	100 hyaline and 5 fatty casts per LPF
Crystals	None
FENa calculated at 2.3%

The patient’s primary care tries to call her again and is able to reach her. The patient reports over the phone that she ran out of her loop diuretic several months ago. She also notes that she has been experiencing sharp right sided abdominal pain and feels like she might have a fever. You advise her to go the emergency department.

Sarah Williams arrives at the emergency department and is found to have acute cholecystitis. She has a cholecystectomy the next day. She has urine studies performed during this admission that show a GFR of 17, specific gravity 1.015, FeNa 2.7%, pH 7, 3+ protein and 1+ blood. Renal ultrasound was unremarkable. Post void residual is normal. A 24-hour urine collection is done while the patient was admitted that showed greater than 4.1 grams of protein. While admitted, it was noted that the patient had significant dependent edema. She continued to complain of feeling short of breath during admission and required 2L oxygen nasal cannula intermittently. She was given 2 mg bumetanide daily and her symptoms had improved by the time she was discharged. She is instructed to follow up with a nephrologist within the next week.

She is started on 40 mg KCl once a day until follow up with her nephrologist. She was re-prescribed furosemide to take at home. All of her other home medications are continued.

The patient follows up with a nephrologist several months later. She complains of hair loss on her legs and under arms. She has had improved energy and a good appetite. She denies fever, chills, flank pain, dysuria or hematuria. She notes she recently switched to a new primary care who discontinued her atorvastatin. She also recently quit smoking. She states she’s been compliant with her medications. Records review indicates that she has not refilled any of her prescriptions since she was discharged from the hospital.

Physical exam

Stat	Value
BP	190/83
HR	77
T	97.8
RR	15
SpO2	98%

Grossly normal physical exam aside from 1-2+ pitting edema to her bilateral lower extremities.

A CMP, PO4, CBC, ANA, C3/C4, hepatitis panel and urine protein electrophoresis are ordered. Her nephrologist decides to obtain an ultrasound guided needle biopsy for definitive diagnosis. The patient is started on amlodipine 5 mg daily. Her furosemide is increased to 60 mg daily.

Her biopsy results are as follows:

Light microscopy: Sections show renal cortical and medullary tissue containing approximately 25 glomeruli, none of which are globally sclerotic. The glomeruli are normal in size with focal, segmental, mesangial matrix expansion and very mild segmental sclerosis. There is no significant tubular atrophy or interstitial fibrosis. The arterioles are unremarkable. 

Immunofluorescence: Sections examined contain 7 glomeruli per level. These show 3-4+ diffuse granular IgG in the peripheral capillary loops. There is 1+ diffuse linear fibrinogen in the loops. IgA, IgM, C3 and C1Q are negative.

Electron microscopy:

The nephrologist informs the patient of her diagnosis and starts her on cyclosporine.