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Large-vessel involvement in granulomatosis with polyangiitis successfully treated with rituximab: A case report and literature review.
- Source :
-
Modern rheumatology [Mod Rheumatol] 2017 Jul; Vol. 27 (4), pp. 699-704. Date of Electronic Publication: 2015 Apr 02. - Publication Year :
- 2017
-
Abstract
- Granulomatosis with polyangiitis (GPA) is primary necrotizing vasculitis, which predominantly affects small to medium vessels. Herein, we describe a case of a 60-year-old female with GPA who developed inflammatory wall thickening localized in the aortic arch, upper abdominal aorta, and pulmonary artery. The wall thickening in the large vessels and other GPA lesions such as lung nodules and orbital mass had failed to respond to high-dose glucocorticoids combined with cyclophosphamide; however, all were successfully treated with rituximab. Our literature review identified 24 cases of large-vessel involvement associated with GPA. Luminal stenosis, occlusion, or wall thickening were observed in 8, periaortitis in 11, and aneurysms in 5 cases. The most commonly affected vessel was the abdominal aorta (12 cases), followed by the thoracic aorta (6 cases), subclavian artery (4 cases), and internal carotid artery (4 cases). Glucocorticoids were used in 23 cases, 20 of which received combination therapy with cyclophosphamide. Surgical or endovascular therapies were performed in 10 cases with aneurysmal dilatation. This is the first case showing the potential efficacy of rituximab for refractory large-vessel involvement associated with GPA.
- Subjects :
- Cyclophosphamide therapeutic use
Female
Glucocorticoids therapeutic use
Granulomatosis with Polyangiitis complications
Humans
Middle Aged
Retreatment
Treatment Outcome
Vascular Diseases complications
Granulomatosis with Polyangiitis drug therapy
Rituximab therapeutic use
Vascular Diseases drug therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1439-7609
- Volume :
- 27
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Modern rheumatology
- Publication Type :
- Academic Journal
- Accession number :
- 25736357
- Full Text :
- https://doi.org/10.3109/14397595.2015.1021950