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

Mihran Naljayan
Mihran NaljayanCase Presenter | Louisiana State University, New Orleans, LA, USA | No conflict of interest reported
Helmut Rennke
Helmut RennkeNephropathology Discussant | Brigham Women's Hospital, Boston, MA, USA | No conflict of interest reported
Ali Poyan Mehr
Ali Poyan MehrNephrology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | Conflict of interest reported: Mallinckrodt - Glomerular Disease Consultant and Research Grant

Case

Patient is a 65-year-old male with long standing hypertension who presents to the hospital with a headache, elevated blood pressure, and anasarca.

About 2 months prior to this presentation he was seen for headaches and elevated BP, and he was noted to have an elevated creatinine of 5 mg/dl. He reported difficulty sleeping the past several months and has noted increased swelling in lower and upper extremities.  He was started on antihypertensives including a diuretic. Unfortunately, he did not follow up for refills on his medications, nor did he present for the urgent renal appointment he was referred to.

This time, he noted a few days before coming in, his blood pressure to be at 220s/160s which prompted him to come to the emergency room.

On presentation, patient reports fatigue and edema. He denies any metallic taste, pruritus, chest pain, nausea, or vomiting.  He has had some decreased appetite.  BP was severely elevated and he was admitted to the ICU for intensive control of BP. Notably, he also had acute kidney injury.

Given patient’s advanced CKD, some uremic symptoms, and significant anasarca, counseling on possible need for renal replacement therapy was initiated in the hospital. Peritoneal dialysis was considered as a preferred option.

In the interim, while following medical management, including volume and BP control, a biopsy was performed (reported below).

Medical history:
Metabolic syndrome
Pneumonia
Hypertension
BPH

Medications:
Furosemide, amlodipine, labetalol, pantoprazol, vitamin D

Physical Exam:
Obese. Initial BP 220/160, anasarca, including upper extremity and facial edema.
Fundoscopic exam with papilledema and microhemorrhages.
4+ lower extremity edema

Laboratory and other data:
Presenting Labs on current hospitalization:
Serum creatinine between 7 to 10 mg/dl during the hospitalization.
SPEP/UPEP negative
Anti-GBM, ANA, cANCA, pANCA negative
ASO titer <100. Complement normal
K/L 4.30 (mildly elevated in light of CKD)
Kappa 170 , Lambda 35
PTH 250
TSH normal
Detailed infectious workup negative
HepB surface Ag and Ab: negative, core IgM negative, core IgG positive (!)
UA with trace blood, >500 protein
Urine protein to creatinine ratio >10 g/g
Review of previous labs:
Serum creatinine one year prior to hospitalization 1.4 mg/dl and UA protein 500
Serum creatinine 3 to 5 months prior to hospitalization ~5 mg/dl, and UA protein >500

Short term follow up:
Dialysis initiated, and ACEi started at discharge, and furosemide 160 mg PO BID.  1 week later seen in clinic still with some edema, improvement in BP, but still elevated.  Renal function stable (residual urine).  Started on metolazone 5 mg daily.

Kidney Pathology

Pathology images pending

Diffuse proliferative (endocapillary) glomerulonephritis. Subendothelial immune complex deposition with a fibrillary structure. No crescent formation. Global glomerulosclerosis in ~50% of glomeruli

Moderate tubular atrophy and interstitial fibrosis. Mild arteriosclerosis and arteriosclerosis. Acute tubulitis. IF: There is peripheral granular staining with some spill over into the mesangium with IgG (2+), IgM (1-2+), C3 (3+), C1q (2+), Kappa and Lambda (2+) with no staining for IgA. EM: There are occasional subendothelial electron dense deposits which on higher power have a fibrillary appearance. There are no subepithelial deposits.

Questions posed & summary of key discussion points

1. What are the differential diagnoses for these biopsy findings and next management options?

Author(s) of case summary:

Case summary pending

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