Membranoproliferative Glomerulonephritis: A Rare Presentation in Systemic Sclerosis
- Glomerular Diseases: From Inflammation to Fibrosis - III
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
- Dekmak, Batoul, Albany Medical College, Albany, New York, United States
- Aydin-Ghormoz, Emmanuel Albert, Albany Medical College, Albany, New York, United States
- Hongalgi, Krishnakumar D., Albany Medical College, Albany, New York, United States
- Mehta, Swati, Albany Medical College, Albany, New York, United States
Systemic sclerosis (SSc) is a chronic autoimmune condition that can affect multiple organ systems. Renal involvement in SSc can range from asymptomatic reduction of GFR to life-threatening scleroderma renal crisis (SRC). Membranoproliferative glomerulonephritis (MPGN) is a rare and under-investigated renal manifestation of SSc. MPGN is a histological pattern of glomerular injury consisting of mesangial hypercellularity and proliferation of glomerular capillary walls with subsequent formation of new basement membrane. Although MPGN can be caused by a variety of primary and secondary conditions, descriptions of its association with SSc are limited. We present a unique case of MPGN as a manifestation of systemic sclerosis in an adult patient, highlighting the challenges of diagnosis and management of MPGN in the context of SSc.
A 64-year-old woman with a history of SSc, controlled hypertension, Raynaud's syndrome, and chronic kidney disease presented to our hospital with acute kidney injury and elevated blood pressure. Laboratory tests showed AKI, proteinuria, microscopic hematuria, and low complement levels. He did not meet the Scleroderma Clinical Trials Consortium Scleroderma Renal Crisis Working Group diagnostic criteria for SRC. Serologies were unrevealing for SLE and hepatitis B and C. No paraprotein was detected. A renal biopsy revealed immune complex-mediated glomerulonephritis (ICGN) with an MPGN pattern. The patient was started on mycophenolate mofetil (MMF) and showed improvement in renal function and complement levels.
This case represents, to our knowledge, the second reported case of MPGN associated with SSc. It emphasizes the importance of understanding the various renal manifestations of SSc in patients with this condition and AKI, and the importance of considering diagnoses other than SRC. MPGN can be immune complex-mediated or complement-mediated, with specific complement levels indicating the pathway involved. Treatment depends on the underlying cause and may involve immunosuppressive agents for underlying autoimmune disease, chemotherapy for hematologic malignancies, or antimicrobials for systemic infection. In this case, the patient showed improvement with MMF therapy. Prompt evaluation and intervention are crucial to prevent permanent kidney damage in SSc patients with renal involvement.