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FRI0493 Clinical and Serological Comparative Analysis of Systemic Sclerosis with or without Overlap Syndromes in A Large Brazilian Cohort
  1. C.M. Silva,
  2. V.S. Viana,
  3. S.G. Pasoto,
  4. L.P. Seguro,
  5. D.C.O. Andrade,
  6. E. Bonfá,
  7. P.D. Sampaio-Barros
  1. Division of Rheumatology, Universidade de São Paulo, São Paulo, Brazil


Background There are scarce data comparing clinical and serological features in patients with systemic sclerosis (SSc) and SSc overlap syndromes (SSc-OS) due to the rarity of these associations.

Objectives To analyze clinical and SSc-associated serological profiles including a panel of novel described antinucleolar antibodies (ANoA) in a large cohort of Brazilian patients with SSc and SSc overlap syndromes.

Methods Three-hundred twenty-eight SSc patients classified according to the ACR/EULAR 2013 SSc criteria, attended at the Scleroderma Outpatient Clinic of a tertiary referral university hospital from 2000 to 2011 were enrolled in the study; 32% had diffuse cutaneous SSc and 68% limited cutaneous SSc. Clinical and demographic data were obtained from an electronic register database. Serum samples were analyzed for the presence of antinuclear antibodies (indirect immunofluorescence on HEp-2 cells), antibodies to Scl-70, PM-Scl, RNA-Pol III, CENP-A/CENP-B, and Ro/SS-A (52 and 60 kDa) (ELISA), and antinucleolar antibodies (ANoA) fibrillarin, Ku, Th/To and NOR90 (line blot immunoassay) using commercial available standardized kits.

Results Two-hundred sixty eigth patients were classified as SSc and sixty patients as SSc-OS (14 with systemic lupus erythematosus, 11 with polymyositis, 7 with rheumatoid arthritis, and 35 with Sjogren Syndrome, isolated or combined). Both groups (SSc-O and SSc) were similar regarding mean age at onset, female predominance, disease duration and ethnicity (p>0.05). Concerning clinical features, there were no significant differences related to the occurrence of pitting scars, digital amputation, calcinosis, telangiectasia, interstitial lung disease, pulmonary hypertension, cardiovascular disease, renal crisis (p>0.05). Conversely, SSc-OS patients had lower mean modified Rodnan skin score (5.6±4.24 vs. 10±11.7, p<0,001),but higher percentage of joint (33 vs. 21%, p=0.04) and muscle involvement (22 vs. 8%, p=0.003) compared to the SSc group. There was no difference regarding the frequency of all autoantibodies tested in the two groups (p>0.05), except for the negative association among anti-RNA-Pol III with SSc-OS (p=0.011).

Conclusions This study identified a comparable occurrence of major organ involvement and ischemic lesions in SSc-OS and SSc. This finding is strengthened by the observation of a similar frequency of all scleroderma autoantibodies in both groups and suggests that SSc determines the clinical manifestation in overlap syndromes in spite of a less extensive skin involvement in these patients.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.5863

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