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
Adam Segal
Adam SegalNephrology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported

Case

Patient is a 41-year-old female without past medical history, who was referred for lower extremity edema and proteinuria.

Medications:
Ibuprofen, 1-2 tablets per month

Physical Exam:
1-2+ edema up to 1/3 of shin. Healthy appearing.

Laboratory and other data:
Creatinine 0.5 mg/dl, cholesterol 260-> 316 mg/dl, serum albumin 3.2-> 2.5 g/dl, protein/creatinine 2-3 g/g, serum PLA2R + with titer 1:160.

Short term follow up:
She was placed on ACEi/ARB, furosemide, and statin. A follow-up PLA2R titer was obtained at around 8 months, when her proteinuria appeared to remain persistent and additional support for treatment decision was being looked for. Her PLA2R titer now was 1:20.

Kidney Pathology

Pathology images pending

Membranous glomerulonephritis.

Light Microscopy: Rare globally sclerotic glomeruli, all others show up to mild mesangial prominence.  Membranes are at most minimally thickened, and only occasional finely granular “holes” are seen on Jones stain.  No crescents or segmental sclerosis are noted.

There is minimal interstitial fibrosis and tubular atrophy.  Intact tubulointerstitium is within normal limits.

Immunofluorescence: There is coarse granular peripheral capillary loop staining for IgG (3-4+),  C3 (1+),  Kappa (3+) Lambda (2-3+),  IgG1 and  IgG4 (both 2-3+), IgG3 (1-2+), IgG2 (1+), and PLA2R (1-2+). 0 to trace mesangial IgM is seen.  IgA and C1q are negative. 1+ C3 is seen along tubular membranes and vessels. Albumin and fibrin are negative.

Electron Microscopy: Widespread foot process effacement. Numerous subepithelial electron dense deposits are noted, with some associated spike formation (Stage 1-2). No subendothelial deposits are noted. The mesangial matrix is at most mildly increased and no significant electron dense deposits are seen there.

Questions posed & summary of key discussion points

1. Patient has been reluctant to immunosuppressive therapy.
2. She also has no children, and is actively trying to conceive (worried about her age).
3. Her proteinuria and nephrotic syndrome has slightly worsened over an 8 month observation period, but her PLA2R titer is trending down.
4. In light of her age, the clinical and laboratory findings,  and desire to conceive, what is the best next step?

Authors of case summary:

Case summary pending

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