Skip to main content

Advertisement

SpringerLink
Log in

Successful treatment of a patient with primary Sjögren's syndrome with Rituximab

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

We report the course of a 55-year-old woman, the first patient with primary Sjögren's syndrome and distal renal tubular acidosis but without lymphoma to be treated with B-cell depletion using Rituximab. Rapidly after B-cell depletion, remarkable improvement in xerostomia occurred, while serological findings and tubular acidosis have been unchanged. In labial salivary gland biopsy, lymphocyte infiltration and particularly CD20-positive cells decreased strikingly. Aquaporin 1 (AQP-1) expression in myoepithelial cells was very low before treatment and increased noticeably. Apical AQP-5 in acinus cells likewise increased following Rituximab. In contrast, basolateral NKCC1 was expressed at unchanged intensity before and following Rituximab. The improvement has been sustained and still is most gratifying 10 months after treatment. B-cell depletion may be effective treatment in Sjögren's syndrome. Likewise, it may now be possible to separate the immunologic phenomena in Sjögren's syndrome from the consequences of prolonged hyposalivation when studying the pathophysiology of xerostomia.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

References

  1. Groom J, Kalled SL, Cutler AH et al (2002) Association of BAFF/BLyS overexpression and altered B cell differentiation with Sjögren's syndrome. J Clin Invest 109:59–68

    Article  PubMed  CAS  Google Scholar 

  2. Somer BG, Tsai DE, Downs L, Weinstein B, Shuster SJ (2003) Improvement in Sjögren's syndrome following therapy with Rituximab for marginal zone lymphoma. Arthritis Rheum 49:394–398

    Article  PubMed  Google Scholar 

  3. Cragg MS, Walshe CA, Ivanóv AO, Glennie MJ (2005) The biology of CD20 and its potential as a target for mAb therapy. Curr Dir Autoimmun 8:140–174

    Article  PubMed  CAS  Google Scholar 

  4. Venables PJW (2004) Sjögren's syndrome. Best Pract Res Clin Rheumatol 18:313–329

    PubMed  CAS  Google Scholar 

  5. Cavill D, Waterman SA, Gordon TP (2004) Antibodies raised against the second extracellular loop of the human muscarinic M3 receptor mimic functional autoantibodies in Sjögren's syndrome. Scand J Immunol 59:261–266

    Article  PubMed  CAS  Google Scholar 

  6. Maruyama T, Saito I, Hayashi Y, Kompfner E, Fox RI, Burton DR, Ditzel HJ (2004) Molecular analysis of the human autoantibody response to α-fodrin in Sjögren's syndrome reveals novel apoptosis-induced specificity. Am J Pathol 165:53–61

    PubMed  CAS  Google Scholar 

  7. Turner RJ, Sugiya H (2002) Understanding salivary fluid and protein secretion. Oral Dis 8:3–11

    Article  PubMed  Google Scholar 

  8. Matsuzaki T, Tajika Y, Ablimit A, Aoki T, Hagiwara H, Takata K (2004) Aquaporins in the digestive system. Med Electron Microsc 37:71–80

    Article  PubMed  CAS  Google Scholar 

  9. Beroukas D, Hiscock J, Gannon BJ, Jonsson R, Gordon TP, Waterman SA (2002) Selective down-regulation of aquaporin-1 in salivary glands in primary Sjögren's syndrome. Lab Invest 82:1547–1552

    PubMed  CAS  Google Scholar 

  10. Li J, Ha YM, Kü NY, Choi SY, Lee SJ, Oh SB, Kim JS, Lee JH, Lee EB, Song YW, Park K (2004) Inhibitory effects of autoantibodies on muscarinic receptors in Sjögren's syndrome. Lab Invest 84:1430–1438

    Article  PubMed  CAS  Google Scholar 

  11. Gresz V, Kwon T-H, Gong H, Agre P, Steward MC, King LS, Nielsen S (2004) Immunolocalization of AQP-5 in rat parotid and submandibular salivary glands after stimulation or inhibition of secretion in vivo. Am J Physiol 287:G151–G161

    CAS  Google Scholar 

  12. Ishikawa Y, Inoue N, Zhenfang Y, Nakae Y (2004) Molecular mechanisms and drug development in aquaporin water channel diseases: the translocation of aquaporin-5 from lipid rafts to the apical plasma membrane of parotid glands of normal rats and the impairment of it in diabetic or aged rats. J Pharmacol Sci 96:271–275

    Article  PubMed  CAS  Google Scholar 

  13. Kallenbach M, Duan H, Ring T (2005) Rituximab induced remission in a patient with Wegener's granulomatosis. Nephron Clin Pract 99:c92–c96

    Article  PubMed  CAS  Google Scholar 

  14. Mariette X, Ravaud P, Steinfeld S, Baron G, Goetz J, Hachulla E, Combe B, Puéchal X, Pennec Y, Sauvezie B, Perdriger A, Hayem G, Janin A, Sibilia J (2004) Inefficacy of infliximab in primary Sjögren's syndrome. Arthritis Rheum 50:1270–1276

    Article  PubMed  CAS  Google Scholar 

  15. Pijpe J, Van Imhoff GW, Vissink A, Van der Wal JE, Kluin PM, Spijkervet FKL, Kallenberg CGM, Bootsma H (2004) Changes in salivary gland immunohistology and function after rituximab mono-therapy in a patient with Sjögren's syndrome and associated MALT-lymphoma. Ann Rheum Dis (epub ahead of print) http://Doi:10.1136/ard.2004.030684

Download references

Author information

Authors and Affiliations

  1. Department of Nephrology, Aalborg Hospital, Hobrovej 18-22, 9100, Aalborg, Denmark

    Troels Ring

  2. Department of Hematology, Aalborg Hospital, Hobrovej 18-22, 9100, Aalborg, Denmark

    Maria Kallenbach

  3. The Water and Salt Research Center, Building 233/234, University of Aarhus, 800, Aarhus, Denmark

    Jeppe Prætorius & Søren Nielsen

  4. Department of Pathology, Aalborg Hospital, Hobrovej 18-22, 9100, Aalborg, Denmark

    Birgitte Melgaard

Authors
  1. Troels Ring
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Maria Kallenbach
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jeppe Prætorius
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Søren Nielsen
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Birgitte Melgaard
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Troels Ring.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ring, T., Kallenbach, M., Prætorius, J. et al. Successful treatment of a patient with primary Sjögren's syndrome with Rituximab. Clin Rheumatol 25, 891–894 (2006). https://doi.org/10.1007/s10067-005-0086-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-005-0086-0

Keywords

Search

Navigation