Abstract: SA-PO240
MPGN and Sarcoidosis: A Rare Presentation
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Mirza, Alamgir, eastern virginia medical school, Norfolk, Virginia, United States
- Magoon, Sandeep, eastern virginia medical school, Norfolk, Virginia, United States
Introduction
Sarcoidosis is a chronic multi-system disease characterized by non-caseating granulomas. It can affect kidneys, but glomerular involvement is rare. There are few case reports of sarcoidosis with membranoproliferative glomerulonephritis (MPGN) where the causality was not proven clearly. Last case described in literature was in 1986. To best of our knowledge, our case is the first in which MPGN is causally related to Sarcoidosis, presented with rapidly progressive glomerulonephritis and severe AKI and first one to be reported since the new classification for MPGN has been proposed.
Case Description
A 32 years old African American male with past medical history of bilateral knee arthritis presented to the hospital with bilateral ankle edema and rash in lower extremities. He was in acute kidney injury (AKI) with creatinine of 3.5 mg/dL. Creatinine 2 years ago was 1.3 mg/dL. Urinalysis showed proteinuria, hematuria, no red blood cell cast. Proteinuia was 6.2 gm/day. Hepatitis B and C, and HIV tests were negative. Cryoglobulin and paraprotein studies were negative. ANA was positive at 1:40, other autoimmune serologies and complements were negative. Angiotensin converting enzyme was elevated at 92 U/L. Renal ultrasound was unremarkable. CT chest showed bilateral hilar lymphadenopathy. Renal biopsy showed MPGN of uncertain etiology. Immunofluorescence (IF) was positive for IgG, IgA, IgM, C3, C1q deposits. Creatinine improved from a peak of 7.4 mg/dL to 3.4 mg/dL with pulse steroids and was discharged on prednisone. A week later he presented back with worsening renal function and was started on dialysis. Lymph node biopsy revealed non caseating granulomas and no infection. Bone marrow biopsy was negative. Cellcept was added to his regimen. His interdylactic rise of creatinine is decreasing. Renal recovery is anticipated.
Discussion
Patient's presentation with arthritis and rash raised suspicion of sarcoidosis related vasculitis supported by a "full house" on IF in renal biopsy and non caseating granulomas on lymph node biopsy. Work up for lymphoma, paraproteinemia, infection and other autoimmune diseases were negative.
In Summary, MPGN with Sarcoidosis is very rare. However when no other etiology is identified, Sarcoidosis should be considered in the differential. There is no consensus on treatment. Steroid is treatment of choice. Aggressive therapy, namely Rituximab, cellcept, plasmapheresis should be considered in severe AKI.