Abstract: FR-PO626
A Case of Histiocytic Glomerulopathy With Features of Thrombotic Microangiopathy With Features of Atypical Sarcoidosis and Lupus Anticoagulant
Session Information
- Glomerular Diseases: Lupus and Vasculitis
November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1302 Glomerular Diseases: Immunology and Inflammation
Authors
- Alom, Md Saiful, Strong Memorial Hospital, Rochester, New York, United States
- Moore, Catherine A., Strong Memorial Hospital, Rochester, New York, United States
- Choung, Hae Yoon Grace, Strong Memorial Hospital, Rochester, New York, United States
Introduction
Hemophagocytic Syndrome (HPS) is a rare, potentially fatal disease, characterized by unrestrained immune activation resulting in hypercytokinemia and multiorgan failure
Case Description
We present a case with subacute progressive renal dysfunction, hematuria and proteinuria in a 73-year-old female with a congenital solitary kidney, hypertension, and primary hyperparathyroidism. She was admitted with serum creatinine (s.cr) of 4.1 g/dl up from baseline (0.9 mg/dl). Additional findings included pancytopenia, hypermetabolic lymphadenopathy, mild hypercalcemia, and fatigue. She underwent extensive work up including PET scan and biopsies of the kidney, lymph node and bone marrow. Biopsy of the kidney showed histiocytic glomerulopathy with features of thrombotic microangiopathy (TMA) while LN exhibited non-necrotizing granulomas typical of sarcoidosis. Bone marrow was diagnosed clonal hematopoiesis of indeterminate potential (CHIP).
Laboratory values of note included positive IgM for Beta-2-glycoprotein and anti-cardiolipin, random urine total protein/creatinine ratio(UPCR) 2.2 gm/gm, soluble IL2 receptor 1652 U/mL, CRP 156 mg/L, LDH 365 U/L, CPK 294, Ferritin 618 ng/ml, and normal serum protein electrophoresis and serum immunofixation. 1,25 Di-Hydroxy Vitamin D was normal
She was placed on prednisone with initial robust improvement in renal parameters: s.cr decrease to 2.1 mg/dL and UPCR to 660 mg/gm. Course was complicated by legionella pneumonia leading to reduction in steroids, and she initiated infliximab infusions. Unfortunately renal function failed to improve with infliximab and s.cr increased to 5 mg/dL
Discussion
Histiocytic glomerulopathy (HG) has been associated with Macrophage Activation Syndrome/HPS following viral infections or hematologic malignancies. Glomerular involvement is rare and usually manifests as either a podocytopathy, TMA, or rarely HG. HG has not been reported with sarcoidosis. The pathogenic role of her positive lupus anticoagulant, given the features of TMA on renal biopsy, and CHIP on LN biopsy is an additional factor under consideration. Findings of CHIP may indicate worse renal prognosis; this association needs further study.