1. The Clinical Relevance of Antifibrillarin (anti-U3- RNP) Autoantibodies in Systemic Sclerosis.
- Author
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Tall, F., Dechomet, M., Riviere, S., Cottin, V., Ballot, E., Tiev, K. P., Montin, R., Morin, C., Chantran, Y., Grange, C., Jullien, D., Ninet, J., Chretien, P., Cabane, J., Fabien, N., and Johanet, C.
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AUTOANTIBODIES , *AUTOIMMUNITY , *SYSTEMIC scleroderma , *IMMUNOGLOBULINS , *CRYOGLOBULINS - Abstract
Systemic sclerosis ( SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of antifibrillarin autoantibodies ( AFA) in patients with SSc. The clinical features of 37 patients with SSc positive for AFA ( AFA+) and 139 SSc patients without AFA ( AFA−) were collected retrospectively from medical records to enable a comparison between AFA− and AFA+ patients. Antifibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Comparing AFA+ and AFA− patients, AFA+ patients were significantly younger at disease onset (36.9 versus 42.9; P = 0.02), more frequently male ( P = 0.02) and of Afro-Caribbean descent (65% versus 7.7%; P < 0.001). At diagnosis, the Rodnan skin score evaluating the cutaneous manifestations was higher (13.3 versus 8.7; P = 0.01) and myositis was also more common in the AFA+ group (31.4% versus 12.2%; P < 0.01). Patients with AFA+ were not associated with diffuse cutaneous SSc or with lung involvement and no difference in survival was observed. Antifibrillarin autoantibodies are associated with patients of Afro-Caribbean origin and can identify patients with SSc who are younger at disease onset and display a higher prevalence of myositis. [ABSTRACT FROM AUTHOR]
- Published
- 2017
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