Systemic sclerosis: demographic, clinical and serological features in 100 Iranian patients

• Published in: Rheumatology international 2013-08, Vol.33 (8), p.1943-1950
• Main Authors: Poormoghim, Hadi, Moghadam, Alireza Salek, Moradi-Lakeh, Maziar, Jafarzadeh, Mehrzad, Asadifar, Behnam, Ghelman, Mohsen, Andalib, Elham
• Format: Article
• Language: English
• Subjects: Adult; Aged; Antibodies, Antinuclear - blood; Antibodies, Antinuclear - immunology; Autoantibodies - blood; Autoantibodies - immunology; Female; Humans; Iran; Male; Medicine; Medicine & Public Health; Middle Aged; Original Article; Rheumatology; Scleroderma, Systemic - blood; Scleroderma, Systemic - immunology
• ISSN: 0172-8172; 1437-160X
• DOI: 10.1007/s00296-013-2668-5

To evaluate demographic, clinical and laboratory features associated with scleroderma-specific auto-antibodies. Sera of 100 patients with systemic sclerosis (SSc) were analyzed by an indirect immunofluorescence technique with HEp-2 cells as a substrate. Specific ANA such as anti-centromere antibodies (ACA), anti-topoisomerase (TOPO), anti-RNA polymerase III (Pol 3), anti-U3-RNP (U3-RNP), anti-Th/To (Th/To) and anti-PM/Scl (PM/Scl) were detected by line immunoassay and anti-U1-RNP (U1-RNP) by ELISA. Frequency of clinical features associated with a specific antibody group was reported cumulatively over the follow-up period. Frequency of specific clinical features was compared across the two disease subtype including limited cutaneous (lcSSc) or diffuse cutaneous (dcSSc) as well as the auto-antibody groups. Ninety-four percent of patients were ANA positive with significant higher skin score, Raynauds and digital ulcer/gangrene. Anti-TOPO was detected in 71 % of all patients, in 90.5 % of dcSSC and in 65.8 % of lcSSc. Anti-TOPO was significantly associated with dcSSc, higher skin score, digital ulcer/gangrene, pulmonary fibrosis, DLCO