A RARE CASE OF PROLIFERATIVE ANCA-NEGATIVE PAUCI-IMMUNE CRESCENTIC GLOMERULONEPHRITIS

Document Type

Conference Proceeding

Publication Date

4-1-2023

Publication Title

Am J Kidney Dis

Abstract

Pauci-immune crescentic glomerulonephritis (PICGN) is a progressive form of GN that is commonly associated with antineutrophil cytoplasmic antibodies (ANCA) vasculitis. Patients with negative ANCA constitute a minority with PICGN. Endocapillary proliferation on histology is uncommon in PICGN. 76-year old female with history of HTN, T2DM presented with petechial rash on her abdomen and extremities. Lab findings showed an elevated SCr. to 1.27 mg/dl. Serology workup revealed negative ANA, ANCA, dsDNA, and cryocrit. Complements (C3 and C4) were normal. Urinalysis was remarkable for microscopic hematuria and albuminuria. Urine PCR resulted 15g/g. Skin biopsy showed leukocytoclastic vasculitis. Her renal function declined with peak SCr. of 5mg/dl within days. Renal biopsy showed PICGN (Figure 1) with an unusual finding of endocapillary proliferation (Figure 2). Isoniazid was initiated for latent tuberculosis with a positive Quantiferon. Additionally, high dose steroids and oral cyclophosphamide were initiated with improvement SCr. to 1.9mg/dl. She never required renal replacement therapy. Presentation of ANCA-negative PICGN is variable and presence of endocapillary proliferation is rare. Aggressive management directed in treating underlying etiologies and immunosuppression can mitigate rapid decline in renal function.

Volume

81

Issue

4

First Page

S41

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