Abstract: TH-PO0839
Vasculitis-Induced Histiocytic Glomerulopathy Without Concurrent Hemophagocytic Lymphohistiocytosis
Session Information
- Glomerular Case Reports: Potpourri
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Rabi, Salma, Division of Nephrology and Hypertension, University of Minnesota, Minneapolis, Minnesota, United States
- Nachman, Patrick H., Division of Nephrology and Hypertension, University of Minnesota, Minneapolis, Minnesota, United States
- Buglioni, Alessia, Mayo Clinic Minnesota Department of Laboratory Medicine and Pathology, Rochester, Minnesota, United States
- Klomjit, Nattawat, Division of Nephrology and Hypertension, University of Minnesota, Minneapolis, Minnesota, United States
Introduction
Histiocytic glomerulopathy is a rare glomerular disorder characterized by foamy lesions and marked macrophage infiltration, often linked to hemophagocytic lymphohistiocytosis (HLH), though rare cases may occur independently of HLH.
Case Description
A 70-year-old woman with hypothyroidism and Raynaud’s presented with 3 weeks of dry cough, low-grade fever, and fingertip discoloration. Azithromycin provided no relief. Labs showed left lower lung infiltrate, leukocytosis, and AKI (Cr 2.58 mg/dL; baseline 0.9). CT ruled out pneumonia, but renal function worsened and digital ischemia progressed to gangrene. Serologies revealed ANA 1:320 (speckled), RF 63 IU/mL, SSA >240 U/mL, low C3/C4, and CRP 242 mg/L. Smith, dsDNA, RNP, ANCA, antiphospholipid, RNA polymerase III Abs and monoclonal protein were negative/normal. UA showed hematuria, pyuria, and urine-protein-creatinine ratio (UPCR) 2.79 g/g. Labs also showed anemia (Hb 9.0s g/dL), thrombocytopenia (40 x10^3/µL), and persistent leukocytosis. Dialysis was initiated. Kidney biopsy revealed histiocytic glomerulopathy with intraluminal foam cells (Figure). HLH was considered but criteria were not met. Findings were also inconsistent with scleroderma. She was diagnosed with severe small vessel vasculitis causing digital gangrene and histiocytic glomerulopathy. She was treated with high-dose prednisone and three IV cyclophosphamide doses (500 mg/m^2), prednisone was tapered over 4.5 months. She improved, came off dialysis within 1 month, and at 5.5 months had Cr 1.99 mg/dL and UPCR 0.19 g/g.
Discussion
We report the first case of vasculitis-associated histiocytic glomerulopathy in the absence of HLH. The patient's favorable response to steroids and cyclophosphamide highlights their potential role in modulating macrophage-driven renal injury.