- Glomerular Diseases: Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
- Ahmed, Marwa, Virginia Commonwealth University Health System, Richmond, Virginia, United States
- Kidd, Jason M., Virginia Commonwealth University Health System, Richmond, Virginia, United States
- Nasr, Samih H., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Bu, Lihong, Mayo Clinic Minnesota, Rochester, Minnesota, United States
Direct glomerular involvement by activated histiocytes is a rare histopathologic finding and may be associated with cryoglobulinemic glomerulonephritis, Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH). We present a case of histiocytic glomerulopathy without a readily identifiable systemic cause.
A 67-year female with recent cholecystectomy presented with abdominal pain and nausea. She was noted to have elevated alkaline phosphatase levels (1000 U/L) but with normal AST/ALT. She subsequently developed anasarca and anuric kidney failure requiring dialysis. Kidney biopsy showed diffuse macrophage foam cells positive for CD68 in glomeruli (image-1). Immunohistochemistry stains for CD1a and Langerin were negative making Langerhans cell histiocytosis unlikely. A bone marrow biopsy showed rare hemophagocytic macrophages. A broad serologic work up was performed and was mostly unremarkable. Although serology showed elevated IL-2 receptor alpha, hyperferritinemia, hypertriglyceridemia, and bone marrow with hemophagocytic macrophages; she did not meet full criteria for HLH. Due to concern for hypersensitivity reaction to metal clips placed during her cholecystectomy, she underwent laparoscopic removal. She was started on high dose methylprednisolone and then oral prednisone. Renal function improved and dialysis was discontinued.
Histiocytic glomerulopathy is a rare entity categorized by the presence of macrophages in glomeruli. We present an idiopathic case which may have been triggered by a hypersensitivity reaction. Treatment options are limited and include steroids, plasmapheresis, and intravenous immunoglobulins (IVIG). Our patient responded to steroids in addition to removal of a potential underlying trigger. Six weeks after her initial presentation she remains off dialysis with excellent renal function.