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Case Reports in Rheumatology
Volume 2016, Article ID 9070487, 6 pages
http://dx.doi.org/10.1155/2016/9070487
Case Report

Pauci-Immune Crescentic Glomerulonephritis in Connective Tissue Disease

Division of Rheumatology, Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA

Received 20 May 2016; Accepted 28 June 2016

Academic Editor: Gregory J. Tsay

Copyright © 2016 Supraja Yeturi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren’s syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus. In this small population of patients there is no correlation with ANCAs. Most of the patients were treated with aggressive immunosuppression and did well if they were treated early in the course of their disease. One of our patients required renal transplant, but she presented late in the course of her disease, as evidenced by chronicity on her renal biopsy. Whether these patients are overlap of vasculitis and other connective tissue diseases or to be considered as a separate entity is yet to be described. Clinicians must be aware of these presentations because initial presentation can be severe.