A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature
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 supporte...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2017/2421760 |
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| author | Richard A. Lau Ramandeep Bains Duminda Suraweera Jane Ma Emil R. Heinze Andrew L. Wong Philip J. Clements |
| author_facet | Richard A. Lau Ramandeep Bains Duminda Suraweera Jane Ma Emil R. Heinze Andrew L. Wong Philip J. Clements |
| author_sort | Richard A. Lau |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-384d737531904bf3b152b9b7a4c4ccc0 |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-384d737531904bf3b152b9b7a4c4ccc02025-02-03T06:08:31ZengWileyCase Reports in Rheumatology2090-68892090-68972017-01-01201710.1155/2017/24217602421760A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the LiteratureRichard A. Lau0Ramandeep Bains1Duminda Suraweera2Jane Ma3Emil R. Heinze4Andrew L. Wong5Philip J. Clements6UCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAUCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAUCLA-Olive View Internal Medicine Program, Department of Medicine, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAUCLA-Olive View Internal Medicine Program, Department of Medicine, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAUCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAUCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAUCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USAThis 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.http://dx.doi.org/10.1155/2017/2421760 |
| spellingShingle | Richard A. Lau Ramandeep Bains Duminda Suraweera Jane Ma Emil R. Heinze Andrew L. Wong Philip J. Clements A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature Case Reports in Rheumatology |
| title | A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature |
| title_full | A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature |
| title_fullStr | A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature |
| title_full_unstemmed | A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature |
| title_short | A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature |
| title_sort | rare case of digital ischemia and gangrene in anca associated vasculitis with review of the literature |
| url | http://dx.doi.org/10.1155/2017/2421760 |
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