Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Anticentromere Antibodies (ACA)-Positive Systemic Sclerosis (SSc) Patients with Sjgren's Syndrome: A Distintict Subset at High Risk of Lymphoma.

Baldini,  Chiara, Grossi,  Sara, Della Rossa,  Alessandra, Luciano,  Nicoletta, Doveri,  Marica, Gaetano Tavoni,  Antonio, Bombardieri,  Stefano


Sjögren's syndrome (SS) has been considerably associated with anticentromere positive (ACA)-limited cutaneous subtype Systemic Sclerosis (SSc). Nonetheless, only a few studies have analysed the pattern of clinical expression and disease evolution in ACA-SSc-SS patients.


to compare the disease phenotype and clinical evolution of ACA-SSc-sSS patients with (a) patients with ACA-SSc and no SS (ACA-SSc) and (b) patients with primary SS (pSS), in order to verify whether the concomitant association between SS and SSc influence the patients' clinical presentation and outcome.

Patients and Methods:

Seventy-eight ACA-SSc-sSS patients (78 F: 0 M; mean age = 62 ± 12, yrs; mean follow-up = 5.4±6, yrs), 127 ACA-SSc patients without SS (119 F: 8 M; mean age = 60 ± 13, yrs; mean follow-up = 4.9±6, yrs), and 374 patients with primary SS (pSS) (363F:11M; mean age 58± 14, years; mean follow-up = 7.3±6.6 years) who had attended our Unit between 1989 and 2009 were included in the study. Diagnosis of SSc was based on the ARA criteria, while diagnosis of pSS and sSS on AECG criteria. Categorical data were compared using the chi-square test and continuous variables by the Student t-test.


(a)When compared to ACA-SSc, ACA-SSc-sSS patients presented a higher frequency of arthralgias (p<0.0001), peripheral nervous involvement (p=0.006), and altered laboratory findings (p<0.0001) (i.e leukopenia, hypocomplementemia, hypergammaglobulinemia, anti-Ro/SSA, anti-La/SSB, Rheumatoid Factor), in addition to the expected increase of symptoms suggestive for glandular involvement. They also presented a lower frequency of pulmonary hypertension (p= 0.02) and sclerodactyly (p<0.0001) with 26/78 ACA-SSc-sSS patients versus 11/127 ACA-SSc patients being classified as "sine scleroderma" (p<0.0001). Noteworthy, ACA-SSc-sSS also presented a higher frequency of MALT lymphomas (p=0.0009) which occurred after a mean disease duration of 19±11 yrs, and was not correlated to skin sclerosis. (b) When compared to pSS patients, ACA-SSc-sSS patients presented a higher frequency of signs and symptoms belonging to the spectrum of SSc (p<0.0001), i.e Raynaud's phenomenon, dysphagia, teleangectasias, heart involvement and pulmonary hypertension, but also an increased prevalence of salivary glands enlargement (p<0.0001). Altered laboratory findings were also more frequent in pSS patients while no differences were observed for lymphoproliferative disorders (p=0.09)


Patients with ACA-SSc-sSS presented features from both diseases: we observed that the presence of SS in ACA-SSc dramatically increased the risk of lymphoma. A careful work up is thus mandatory in the follow-up of ACA-SSc-sSS patients.

To cite this abstract, please use the following information:
Baldini, Chiara, Grossi, Sara, Della Rossa, Alessandra, Luciano, Nicoletta, Doveri, Marica, Gaetano Tavoni, Antonio, et al; Anticentromere Antibodies (ACA)-Positive Systemic Sclerosis (SSc) Patients with Sjgren's Syndrome: A Distintict Subset at High Risk of Lymphoma. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1884
DOI: 10.1002/art.29649

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