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

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Abstract

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 <70 %. U1-RNP antibody was associated with lower fibrosis in lung. ACA was positive in 7 % of patients and exclusively in those with lcSSc. We did not find association between gender and presence of auto-antibodies. Anti-TOPO antibody had a high prevalence in contrast to low prevalence of ACA antibody. There were no differences in clinical subtypes of the disease in patients with positive anti-TOPO and positive ACA. Differences in prevalence of auto-antibodies are suggestive of further genetic study.

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Acknowledgments

This research has been funded by Tehran University of Medical Sciences., Immunology Research Center.

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Authors and Affiliations

  1. Rheumatology Research Center, Firoozgar Hospital Tehran, Tehran University of Medical Sciences, Tehran, Iran

    Hadi Poormoghim

  2. Immunology Research Center, Tehran University of Medical Sciences, Tehran, Iran

    Alireza Salek Moghadam, Maziar Moradi-Lakeh, Mehrzad Jafarzadeh, Behnam Asadifar, Mohsen Ghelman & Elham Andalib

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  1. Hadi Poormoghim
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  2. Alireza Salek Moghadam
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Correspondence to Hadi Poormoghim.

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Poormoghim, H., Moghadam, A.S., Moradi-Lakeh, M. et al. Systemic sclerosis: demographic, clinical and serological features in 100 Iranian patients. Rheumatol Int 33, 1943–1950 (2013). https://doi.org/10.1007/s00296-013-2668-5

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  • DOI: https://doi.org/10.1007/s00296-013-2668-5

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