Abstract
Primary Sjogren’s syndrome (pSS) is a chronic autoimmune disorder of the exocrine glands. The diagnosis is largely based on keratoconjunctivitis sicca and xerostomia in the presence of anti-SS-A and/or SS-B antibodies. Anticentromere antibodies (ACA) have occasionally been reported in patients with pSS. We describe two patients with pSS associated with ACA, initially diagnosed as limited systemic sclerosis. Symptoms at the time of initial presentation were dry eyes and mouth, arthralgias, and Raynaud’s phenomenon. Both patients developed small vessel cutaneous vasculitis, parotid enlargement, low C4 complement levels, positive rheumatoid factor, and lymphoma. These findings suggest that patients with pSS who have ACA may be a subgroup of patients at increased risk of extraglandular systemic manifestations and lymphoma.
This is a preview of subscription content, log in via an institution to check access.
References
Routsias JG, Tzioufas AG (2007) Sjogren’s syndrome—study of autoantigens and autoantibodies. Clin Rev Allergy Immunol 32:238–251
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH (2002) Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554–558
Salliot C, Gottenberg JE, Bengoufa D, Desmoulins F, Miceli-Richard C, Mariette X (2007) Anticentromere antibodies identify patients with Sjögren’s syndrome and autoimmune overlap syndrome. J Rheumatol 34:2253–2258
Fox RI (2005) Sjogren’s syndrome. Lancet 366:321–331
Vlachoyiannopoulos PG, Drosos AA, Wiik A, Moutsopoulos HM (1993) Patients with anticentromere antibodies, clinical features, diagnoses and evolution. Br J Rheumatol 32:297–301
Caramaschi P, Biasi D, Carletto A, Manzo T, Randon M, Zeminian S, Bambara LM (1997) Sjogren’s syndrome with anticentromere antibodies. Rev Rhum Engl Ed 64:785–788
Ramos-Casals M, Nardi N, Brito-Zeron P et al (2006) Atypical autoantibodies in patients with primary Sjogren’s síndrome: clinical characteristics and follow –up of 82 cases. Semin Arthritis Rheum 35:312–321
Hsu TC, Chang CH, Lin MC, Liu ST, Yen TJ, Tsay GJ (2006) Anti-CENP-H antibodies in patients with Sjogren’s syndrome. Rheumatol Int 26:298–303
Katano K, Kawano M, Koni I, Sugai S, Muro Y (2001) Clinical and laboratory features of anticentromere antibody positive primary Sjogren’s Syndrome. J Rheumatol 28:2238–2244
Voulgarelis M, Tzioufas AG, Moutsopoulos HM (2008) Mortality in Sjögren’s syndrome. Clin Exp Rheumatol 26:S66–S71
Kassan SS, Thomas TL, Moustsoupoulos HM et al (1978) Increased risk of lymphoma in sicca syndrome. Ann Intern Med 89:888–892
Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacbosson LT (2006) Lymphoma and other malignancies in primary Sjögren’s syndrome A cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 65:796–803
Guyomard S, Salles G, Coudurier M et al (2003) Prevalence and pattern of antinuclear autoantibodies in 347 patients with non-Hodgkin’s lymphoma. Br J Hematol 122:1–10
Bencimon C, Salles G, Moreira A, Guyomard S, Coiffier B, Bienvenu J, Fabien N (2005) Prevalence of anticentromere F protein autoantibodies in 347 patients with non-Hodgkin’s lymphoma. Ann N Y Acad Sci 1050:319–326
Disclosures
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Gulati, D., Kushner, I., File, E. et al. Primary Sjogren’s syndrome with anticentromere antibodies—a clinically distinct subset. Clin Rheumatol 29, 789–791 (2010). https://doi.org/10.1007/s10067-009-1359-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-009-1359-9