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Kidney Week

Abstract: TH-PO553

Double Trouble or a Separate Entity: AKI Secondary to ANCA Vasculitis with IgG4 Nephropathy

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Mamlouk, Omar, The University of Texas Health Science Center at Houston, Houston, Texas, United States
  • Khairalla, Hanan A., The University of Texas Health Science Center at Houston, Houston, Texas, United States
  • Glass, William F., The University of Texas Health Science Center at Houston, Houston, Texas, United States
  • Khattak, Aisha, The University of Texas Health Science Center at Houston, Houston, Texas, United States
Introduction

Antineutrophil Cytoplasmic Antibody-Associated (ANCA) vasculitis is reported to be associated with tissue infiltration by IgG4 cells and IgG4-related disease (IgG4-RD) is associated with elevated ANCA titers. IgG4-RD diagnosis is considered with caution in a patient with ANCA vasculitis as JSN criteria, one of widely used criteria for diagnosis, suggests that IgG4-RD is an unlikely diagnosis in patient with vasculitis. However, there is growing evidence of possible overlap between IgG4-RD and ANCA vasculitis, especially granulomatosis with polyangiitis ,GPA. Few case reports showed histological evidence of both disease in the same organ. We present a case of GPA disease with concurrent histological evidence suggestive of IGg4 disease involving the kidney. These patients when treated with rituximab had good disease outcome

Case Description

A 61 year old woman with multiple co-morbidities including hypertension, chronic obstructive lung disease and chronic kidney disease was admitted for evaluation of shortness of breath and dysuria. she developed acute kidney injury during the first week of hospitalization along with purpuric rash on legs. no ocular, thyroid, salivary or oral manifestation by history or examination. urine analysis showed no dysmorphic RBCs. UPC was of 1.5. Serology for ANA, Anti-dsDNA, hepatitis panel and HIV was negative. PR3-ANCA was + and C3&4 were low. Renal biopsy was performed with finding of acute focal segmental necrotizing pauci-immune glomerulonephritis, active tubulointerstitial nephritis with interstitial and tubular basement membrane immune complex deposits ,area of storiform fibrosis , and area of abundant IgG4 (20 cells/HPF). Serum IgG4 subclass level was elevated. These findings are suggestive of IgG4-RD. A CT of abdomen/pelvis did not reveal any other organ involvement. she was started on prednisone and rituximab with improvement of renal function back to baseline

Discussion

Clinical & histological evidence suggestive of IgG4-RD and ANCA vasculitis can co-exist in the same organ, including the kidney. Our findings support the growing evidence of possible association between IgG4-RD and GPA vasculitis. Understanding this association might help us have better understanding of underlying pathophysiology of both diseases. Rituximab was observed to be effective for treatment of IgG4-RD overlapping with ANCA vasculitis