We’re sorry, something doesn't seem to be working properly.

Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

We’re sorry, something doesn't seem to be working properly.

Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Advertisement

Clinical and serologic features of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia: a large-scale cross-sectional study

We’re sorry, something doesn't seem to be working properly.

Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

  • 489 Accesses

  • 7 Citations

Abstract

Autoimmune hemolytic anemia (AIHA) is an uncommon but clinically significant disorder in primary Sjögren’s syndrome (pSS). Among 565 pSS patients hospitalized in Peking University People’s Hospital from January 2000 to March 2013, 16 patients were diagnosed with AIHA (2.8 % prevalence). AIHA presented at the onset of pSS without overt sicca symptoms in 3 of the 16 patients. Primary biliary cirrhosis (PBC) was more prevalent in the patients with SS-AIHA than in those without (p = 0.007). Edema, fever, and liver involvement occurred significantly more frequently in pSS patients with AIHA than those without AIHA (p = 0.035, p = 0.029, p = 0.024, respectively). The pSS patients with AIHA were more vulnerable to leukopenia and thrombocytopenia than those without AIHA (p = 0.004 and p = 0.001, respectively). Additionally, the levels of complement component 3 (C3) and complement component 4 (C4) were significantly lower in the SS-AIHA group (p = 0.008 and p = 0.037, respectively). Taken together, our results indicate that pSS should be considered in the differential diagnosis of AIHA, even in the absence of sicca symptoms. Among pSS patients, the existence of PBC, cytopenia, or hypocomplementemia suggests a higher risk of suffering from AIHA.

This is a preview of subscription content, log in to check access.

Access options

Buy single article

Instant unlimited access to the full article PDF.

US$ 39.95

Price includes VAT for USA

Subscribe to journal

Immediate online access to all issues from 2019. Subscription will auto renew annually.

US$ 99

This is the net price. Taxes to be calculated in checkout.

References

  1. 1.

    Tincani A, Andreoli L, Cavazzana I, Doria A, Favero M, Fenini MG et al (2013) Novel aspects of Sjogren’s syndrome in 2012. BMC Med 11:93

  2. 2.

    Tzioufas AG, Kapsogeorgou EK, Moutsopoulos HM (2012) Pathogenesis of Sjogren’s syndrome: what we know and what we should learn. J Autoimmun 39:4–8

  3. 3.

    Gehrs BC, Friedberg RC (2002) Autoimmune hemolytic anemia. Am J Hematol 69:258–71

  4. 4.

    Schattner A, Shtalrid M, Berrebi A (1983) Autoimmune hemolytic anemia preceding Sjogren’s syndrome. J Rheumatol 10:482–4

  5. 5.

    Aoki A, Ohno S, Ueda A, Ideguchi H, Ohkubo T, Hagiwara E et al (2000) Hematological abnormalities of primary Sjogren’s syndrome. Nihon Rinsho Meneki Gakkai Kaishi 23:124–8

  6. 6.

    Zhou JG, Qing YF, Jiang L, Yang QB, Luo WF (2010) Clinical analysis of primary Sjogren’s syndrome complicating anemia. Clin Rheumatol 29:525–9

  7. 7.

    Zhang S, Wu X (2013) Clinical analysis on 28 cases of primary Sjogren’s syndrome complicated with anemia. Journal of Hematology(China) 26:40–41

  8. 8.

    Shimoji S, Takiuchi Y, Maruoka H, Inoue D, Kimura T, Mori M et al (2011) Exacerbation of autoimmune neutropenia to agranulocytosis in association with severe autoimmune thrombocytopenia and hemolytic anemia in a patient with Sjogren’s syndrome. Rinsho Ketsueki 52:535–9

  9. 9.

    Boling EP, Wen J, Reveille JD, Bias WB, Chused TM, Arnett FC (1983) Primary Sjogren’s syndrome and autoimmune hemolytic anemia in sisters. A family study. Am J Med 74:1066–71

  10. 10.

    Yamanouchi J, Yokota E, Yamauchi Y, Matsumoto I (1998) A case of Sjogren’s syndrome associated with advanced hemolytic anemia caused by worsening autoimmune cholangiopathy. Ryumachi 38:595–9

  11. 11.

    Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE et al (2002) Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554–8

  12. 12.

    Kikawada M, Watanabe D, Kimura A, Hanyu H, Serizawa H, Iwamoto T (2005) Autoimmune hemolytic anemia in an elderly patient with primary Sjogren’s syndrome. Intern Med 44:1312–5

  13. 13.

    Schattner AFJ, Klepfish A, Berrebi A (1983) Immune cytopenias as the presenting finding in primary Sjögren’s syndrome. QJM 93:825–29

  14. 14.

    Tishler M, Yaron I, Shirazi I, Yaron M (2001) Clinical and immunological characteristics of elderly onset Sjogren’s syndrome: a comparison with younger onset disease. J Rheumatol 28:795–7

  15. 15.

    Haga HJ, Jonsson R (1999) The influence of age on disease manifestations and serological characteristics in primary Sjogren’s syndrome. Scand J Rheumatol 28:227–32

  16. 16.

    Alarcon-Segovia D, Diaz-Jouanen E, Fishbein E (1973) Features of Sjogren’s syndrome in primary biliary cirrhosis. Ann Intern Med 79:31–6

  17. 17.

    Assimakopoulos SF, Michalopoulou S, Melachrinou M, Giannakoulas N, Papakonstantinou C, Lekkou A et al (2007) Primary Sjogren syndrome complicated by autoimmune hemolytic anemia and pure red cell aplasia. Am J Med Sci 334:493–6

  18. 18.

    Kokori SI, Ioannidis JP, Voulgarelis M, Tzioufas AG, Moutsopoulos HM (2000) Autoimmune hemolytic anemia in patients with systemic lupus erythematosus. Am J Med 108:198–204

  19. 19.

    Matsumoto T, Morizane T, Aoki Y, Yamasaki S, Nakajima M, Enomoto N et al (2005) Autoimmune hepatitis in primary Sjogren’s syndrome: pathological study of the livers and labial salivary glands in 17 patients with primary Sjogren’s syndrome. Pathol Int 55:70–6

  20. 20.

    Tian Y, Wang C, Liu JX, Wang HH (2009) Primary biliary cirrhosis-related autoimmune hemolytic anemia: three case reports and review of the literature. Case Rep Gastroenterol 3:240–247

  21. 21.

    Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM (2000) Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum 29:296–304

  22. 22.

    Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T et al (2008) Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford) 47:1686–91

  23. 23.

    Lodde BM, Sankar V, Kok MR, Leakan RA, Tak PP, Pillemer SR (2006) Serum lipid levels in Sjogren’s syndrome. Rheumatology (Oxford) 45:481–4

Download references

Acknowledgments

The authors appreciate Miss Jieyi Jiang for her aid in statistical sampling and thank all the fellows and students in Peking University People’s Hospital who contributed to the study. This work was supported by grants from National Sci-Tech Support Program (2014BAI07B01), National Natural Science Foundation of China (81471601), and the Beijing Natural Science Foundation (7152150).

Author information

Correspondence to Zhanguo Li.

Ethics declarations

Disclosures

None.

Additional information

Wen Wen and Yanying Liu contributed equally to this work.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Wen, W., Liu, Y., Zhao, C. et al. Clinical and serologic features of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia: a large-scale cross-sectional study. Clin Rheumatol 34, 1877–1884 (2015). https://doi.org/10.1007/s10067-015-3081-0

Download citation

Keywords

  • Anemia
  • Autoimmunity
  • Cytopenia
  • Primary biliary cirrhosis
  • Sjögren’s syndrome