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

 Jonathan Slater
Jonathan SlaterCase Presenter | Baystate Medical Center, Springfield, MA, USA | No conflict of interest reported
Mihran Naljayan
Mihran NaljayanNephrology Discussant | Louisiana State University Health Sciences Center, New Orleans, LA, USA | No conflict of interest reported
Vanesa Bijol
Vanesa BijolNephropathology Discussant | Brigham and Women's Hospital, Boston, MA, USA | No conflict of interest reported

Case

Patient is a 25-year-old male with hypertension and nephrotic range proteinuria identified approximately one year prior to current presentation.

Medical history:
Hypertension
Hyperlipidemia
Obesity
Ankle fracture due to motor vehicle accident

Family history:
Positive for hypertension in father, and cerebrovascular accident involving his mother.

Medications:
Simvastatin
Lisinopril

Physical Exam:
BMI: 33.3
Blood Pressure: 118/78 mm Hg
No rash, ulcers, or joint effusion.
Unremarkable otherwise

Laboratory and other data:
BUN 12
Creatinine 1.1
Potassium 3.8
Bicarbonate 25
Calcium 9
Phosphorous 3.8
Albumin 4
Hemoglobin 16
Total cholesterol 170
LDL 90
Infectious and serological workup unrevealing.
Urinary albumin to creatinine ratio >6000 mg/g

Short term follow up:
Interim history: Enrollment into research protocol for the study of genetic or acquired forms of C3 GN was considered, however, he did not meet inclusion criteria and the various differentials were debated, including post-infectious versus C3 glomerulopathy.
Workup negative, including HIV, hepB/C, Parvo-virus, no IVDU, no suspicious drugs or medications.

Kidney Pathology

Pathology images pending

First biopsy ~6 months prior to current presentation: Collapsing FSGS. Question raised of ischemic glomerular injury vs. toxin/vasospastic type disease; additionally, isolated C3 deposition suggestive of post infectious GN vs. possibly of C3 Glomerulopathy.

Repeat biopsy ~ months later, at current presentation: Preliminary verbal report: worsening glomerulosclerosis; scattered collapsing FSGS of ischemic pattern, C3G deposits with mesangio-proliferative pattern.

Questions posed & summary of key discussion points

1. What is the possible underlying cause of this patient’s C3 deposits?
2. What is the differential (and significance) for the collapsing FSGS?
3. Would testing for mutations in the alternative pathway components be helpful? And how would this change your management?

Author(s) of case summary:

Case summary pending

If you would like to be a contributing author for the creation of didactic materials and case summaries, please contact us at [email protected].