ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: TH-PO731

Histiocytic Glomerulopathy

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • 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
Introduction

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.

Case Description

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.

Discussion

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.

Image-1