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Kidney Week

Abstract: TH-PO0436

Takayasu Arteritis Associated with Collapsing FSGS

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: Mechanisms, including Podocyte Biology

Authors

  • Abdelwahab, Abeer, University of Khartoum Faculty of Medicine, Khartoum, Sudan
  • Abdallah, Ahmed, Ochsner Health, New Orleans, Louisiana, United States
  • Velez, Juan Carlos Q., Ochsner Health, New Orleans, Louisiana, United States
Introduction

Takayasu arteritis (TA) is a rare granulomatous large-vessel vasculitis affecting the aorta and major branches, most common in young women. Glomerular disease is uncommon. We report TA associated with collapsing focal segmental glomerulosclerosis (FSGS).

Case Description

A 42-year-old woman with TA presented with vomiting, facial edema, and headache one month after stopping methotrexate and adalimumab. Blood pressure was 227/120 mmHg with 2+ LE edema. Labs showed serum creatinine 1.3 mg/dL and urine protein–creatinine ratio 11 g/g, with inactive sediment. Serologies were negative; complement levels were normal. Kidney biopsy demonstrated ischemic changes with mesangiolysis, focal fibrin thrombi, and multiple glomeruli with segmental sclerosis with collapse of the glomerular tuft and 15% IFTA. Immunofluorescence was negative. Electron microscopy showed wrinkled GBM, endothelial swelling, and diffuse podocyte effacement without deposits. Treatment included prednisone, lisinopril, methotrexate, and tocilizumab.

Discussion

Renal involvement in TA usually presents as renovascular hypertension; intrinsic glomerular disease is rare. TA associated FSGS, especially the collapsing variant, is exceedingly uncommon. Proposed mechanisms include ischemia, RAS activation with cytokine mediated podocyte injury (IL 6,VEGF). To our knowledge, this is the fifth reported case of this association and the second with collapsing FSGS.