Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis

Autor: Jayne Moxey, Molla Huq, Susanna Proudman, Joanne Sahhar, Gene-Siew Ngian, Jenny Walker, Gemma Strickland, Michelle Wilson, Laura Ross, Gabor Major, Janet Roddy, Wendy Stevens, Mandana Nikpour
Jazyk: angličtina
Rok vydání: 2019
Předmět:
Zdroj: Arthritis Research & Therapy, Vol 21, Iss 1, Pp 1-12 (2019)
Druh dokumentu: article
ISSN: 1478-6362
DOI: 10.1186/s13075-019-1839-5
Popis: Abstract Background Up to 12% of patients with systemic sclerosis (SSc) have anti-neutrophil cytoplasmic antibodies (ANCA). However, the majority of these patients do not manifest ANCA-associated vasculitis (AAV) and the significance of ANCA in these patients is unclear. The aim of this study is to determine the prevalence of ANCA in a well-characterised SSc cohort and to examine the association between ANCA and SSc clinical characteristics, other autoantibodies, treatments and mortality. Methods Clinical data were obtained from 5 centres in the Australian Scleroderma Cohort Study (ASCS). ANCA positive was defined as the presence of any one or combination of cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA), atypical ANCA, anti-myeloperoxidase (anti-MPO) or anti-proteinase-3 (anti-PR3). Associations of demographic and clinical features with ANCA were investigated by logistic or linear regression. Survival analysis was performed using Kaplan-Meyer curves and Cox regression models. Results Of 1303 patients, 116 (8.9%) were ANCA positive. Anti-PR3 was more common than anti-MPO (13.8% and 11.2% of ANCA-positive patients, respectively). Only 3 ANCA-positive patients had AAV. Anti-Scl-70 was more common in ANCA positive vs ANCA negative (25% vs 12.8%, p
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