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 Twitter

Kidney Week

Abstract: FR-PO626

A Case of Histiocytic Glomerulopathy With Features of Thrombotic Microangiopathy With Features of Atypical Sarcoidosis and Lupus Anticoagulant

Session Information

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.