Abstract
This is a cross-sectional study to assess the prevalence and causes of anemia in the primary Sjögren’s syndrome (pSS). One hundred and thirty-two consecutive patients with pSS were enrolled into the study. Standard hematological and immunological tests and examination of bone marrow were performed. Anemia occurred in 45 (34.1%) patients. The causes of anemia included anemia of chronic disease (69%), autoimmune hemolytic anemia (AIHA, 18%), iron deficiency anemia (9%) and other causes (4%), of which AIHA caused the most severe anemia. The prevalence of ANA, anti-Ro/SSA, and anti-La/SSB was much higher in patients with anemia than those without anemia. Anticardiolipin antibodies were most commonly detected in AIHA; the prevalence of IgG and hypocomplementemia in AIHA was much higher in patients without anemia. Abnormal bone marrow changes were observed in two cases with anemia, one with morphological changes in the myeloid, megakaryocytic, and erythroid lineages and one with hypocellularity in the erythroid lineage. Therefore, pSS patients with anemia may be associated with destruction of peripheral mature blood cells, impaired red cells production, and hematopoietic abnormalities due to an immune mechanism, although the concrete pathogenesis is still unclear.
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References
Fox RI (2005) Sjögren’s syndrome. Lancet 366:321–331
Garcia-Carrasco M, Ramos-Casals M, Rosas J et al (2002) Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 81:270–280
Asmussen K, Andersen V, Bendixen G et al (1996) A new model for classification of disease manifestations in primary Sjögren’s syndrome: evaluation in a retrospective long-term study. J Int Med 239:475–482
Ramakrishna R, Chaudhuri K, Sturgess A et al (1992) Hematological manifestations of primary Sjögren’s syndrome: a clinicopathological study. Q J Med 83:547–554
Ramos-Casals M, Font J, Garcia-Carrasco M et al (2002) Primary Sjögren syndrome: hematologic patterns of disease expression. Medicine (Baltimore) 81:281–292
Okada J (2006) Autoimmune hemolytic anemia in primary Sjögrens syndrome. Intern Med 45:669–670
Bloch KJ, Buchanan WW, Wohl MJ et al (1965) Sjögren’s syndrome: a clinical, pathological and serological study of sixty-two cases. Medicine (Baltimore) 44:187–231
Coppo P, Sibilia J, Maloisel F et al (2003) Primary Sjögren’s syndrome associated agranulocytosis: a benign disorder? Ann Rheum Dis 62:476–478
Vitali C, Bombardieri S, Jonsson R et al (2002) Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group J. Ann Rheum Dis 61:554–558
Erslev AJ (1990) Hematology. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA (eds) Anemia of chronic disorders. New York, pp 540–546
Packman CH, Leddy JP (1990) Hematology. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA (eds) Acquired hemolytic anemia due to warm-reacting autoantibodies. New York, pp 666–675
Erslev AJ (1990) Hematology. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA (eds) Pure red cell aplasia. New York, pp 430–438
Sutcliffe N, Inanc M, Speight P (1998) Predictors of lymphoma development in primary Sjögren’s syndrome. Semin Arthritis Rheum 28:80–87
Skopouli FN, Dafni U, Ioannidis JP et al (2000) Clinical evolution, and morbidity and mortality of Primary Sjögren’s syndrome. Semin Arthritis Rheum 29:296–304
Martinez-Lavin M, Vaughan JH, Tan EM (1979) Autoantibodies and the spectrum of Sjögren’s syndrome. Ann Intern Med 91:185–190
Aoki A, Ohno S, Ueda A et al (2000) Hematological abnormalities of primary Sjögren’s syndrome. Nihon Rinsho Meneki Gakkai Kaishi 23:124–128
Alexander EL, Arnett FC, Provost TT et al (1983) Sjögren’s syndrome: Association of anti-Ro (SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Ann Intern Med 98:155–159
Hammond A, Rudge AC, Loizou S et al (1989) Reduced numbers of complement receptor type 1 on erythrocytes are associated with increased levels of anticardiolipin antibodies. Findings in patients with systemic lupus erythematosus and the antiphosphilipid syndrome. Arthritis Rheum 32:259–264
Szodoray P, Papp G, Horvath IF et al (2009) Cells with regulatory function of the innate and adaptive immune system in primary Sjögren's syndrome. Clin Exp Immunol 157:343–349
Means RT, Krantz SB (1992) Progress in understanding the pathogenesis of the anemia of chronic disease. Blood 80:1639–1647
Means RT Jr, Dessypris EN, Krantz SB (1992) Inhibition of human erythroid colony-forming unit by interleukin-1 is mediated by gamma interferon. J Cell Physiol 150:59–64
Jongen-Lavrencic M, Peeters HR, Vreugdenhil G et al (1995) Interaction of inflammatory cytokines and erythropoietin in iron metabolism and erythropoiesis in anemia of chronic disease. Clin Rheumatol 14:519–525
Hirayama M, Kohgo Y, Kondo H et al (1993) Regulation of iron metabolism in HepG2 cells: a possible role for cytokines in the hepatic deposition of iron. Hepatology 18:874–880
Faquin WC, Schneider TJ, Goldberg MA (1992) Effect of inflammatory cytokines on hypoxia-induced erythropoietin production. Blood 79:1987–1994
Haga HJ, Jonsson R (1999) The influence of age on disease manifestations and serological characteristics in primary Sjögren’s syndrome. Scand Rheumatol 28:227–232
Tishler M, Yaron I, Shirazi I et al (2001) Clinical and immunological characteristics of elderly onset Sjögren’s syndrome: a comparison with younger onset disease. J Rheumatol 28:795–797
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Jing-Guo Zhou and Yu-Feng Qing contributed equally to this work.
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Zhou, JG., Qing, YF., Jiang, L. et al. Clinical analysis of primary Sjögren’s syndrome complicating anemia. Clin Rheumatol 29, 525–529 (2010). https://doi.org/10.1007/s10067-009-1366-x
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DOI: https://doi.org/10.1007/s10067-009-1366-x