To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.

Invited Speakers

Ali Poyan Mehr
Ali Poyan MehrCase Presenter | Beth Israel Deaconess Medical Center, Boston, MA, USA | Conflict of interest reported: Mallinckrodt - Glomerular Disease Consultant and Research Grant
Isaac Stillman
Isaac StillmanNephropathology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported
Adam Segal
Adam SegalNephrology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported

Case

A middle aged female is referred to establish care with a primary care physician for preoperative evaluation for cataracts surgery. She had not seen any care provider for the past 30 years. On initial evaluation by primary care physician she is found to have a serum K of 2.9 mmol/l and sodium 130 mmol/l, and serum bicarbonate 30 mmol/l for which she is referred to renal clinic. In the nephrology clinic she reports  chronic cough, and a weight loss of 15 pounds over the past year, no edema, but nocturia and frothy urine, the duration of which she can’t recall. She drinks daily “few bottles of beer”. Otherwise no rash, joint pain, or other complaints.

Medical history:
Denies any

Social history:
Smokes 1 pack of cigarettes per day (40 years history), and drinks several servings of beer or other alcoholic beverages per day.

Family history:
History is positive for lung cancer (father) and cirrhosis (brother).

Medications:
None. Denies any over the counter medications.

Physical Exam:
Small and thin appearing female, with BP 190/100, no edema, no rash, but several tattoos across upper and lower extremities. No joint abnormalities.

Laboratory and other data:
Persistent leukocytosis with white blood count of 12-15 thousand/microliter, and persistent thrombocytosis with platelets of 400-500 thousands per microliter, serum creatinine 0.7 mg/dl.
On initial presentation, serum potassium of 2.8 mEq/l and sodium 132 mEq/l, bicarbonate 30 mEq/l. Urinary protein to creatinine ratio of 15 g/g and albumin to creatinine ratio of 9000 mg/g. Sediment without any RBCs, WBCs or casts. CT Chest: Chronic pulmonary changes with parenchymal destruction, hyperinflation and scarring most pronounced upper lobes.  Bilateral apical pleural-parenchymal scarring.  Calcified right upper lobe granuloma.  Right upper lobe subpleural nodule or scar measuring less than 4 mm.  Nodule or possibly calcified granuloma inferior right upper lobe measuring less than 4 mm.  No focal consolidation.

Short term follow up:
Proteinuria improved down to 3 grams with antihypertensive therapy (angiotensin receptor blocker and spironolactone). Spironolactone and potassium were discontinued. Patient started on prednisone 50mg daily for 3 months. Weight gain and psycho-emotional side effects led to rapid taper. Now at 5mg with protein to creatinine ratio of 1.5 g/g.

Kidney Pathology

Pathology images pending

Consistent with minimal change-focal and segmental glomerulosclerosis category of renal disease.

Several glomeruli are either globally or segmentally sclerotic depending on the level. There is mild mesangial proliferation and matrix increase with occasional areas of increased proliferation and sclerotic changes . Mild interstitial fibrosis and tubular atrophy is seen.  The arterioles/arteries
show fibrotic changes, and mild intimal fibroplasia.

There is no staining for IgG, IgA, IgM, C3, fibrin, albumin, kappa light chain, lambda light chain or C1q.

Fine structural studies of glomeruli show fairly extensive foot process effacement. No distinct subepithelial, subendothelial, nor mesangial electron dense deposits are seen. These findings are consistent with the minimal change-focal and segmental glomerulosclerosis category of renal disease.

Questions posed & summary of key discussion points

1. Could this be secondary MCD/FSGS, and what other work up should be done?
2. Next steps in therapeutic management?

Authors of case summary:

Case summary pending

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