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Case Rep Rheumatol. 2017;2017:2421760. doi: 10.1155/2017/2421760. Epub 2017 Feb 28.

A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature.

Case reports in rheumatology

Richard A Lau, Ramandeep Bains, Duminda Suraweera, Jane Ma, Emil R Heinze, Andrew L Wong, Philip J Clements

Affiliations

  1. UCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USA.
  2. UCLA-Olive View Internal Medicine Program, Department of Medicine, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USA.
  3. UCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USA; UCLA-Olive View Internal Medicine Program, Department of Medicine, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USA.

PMID: 28348913 PMCID: PMC5350346 DOI: 10.1155/2017/2421760

Abstract

This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases.

Conflict of interest statement

The authors declare that there is no conflict of interests regarding the publication of this paper.

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