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  • Review Article
  • Published:

Treatment of primary Sjögren syndrome

Nature Reviews Rheumatology volume 12, pages 456–471 (2016)Cite this article

Subjects

Key Points

  • Treatment decisions in primary Sjögren syndrome (pSS) are based on the initial evaluation of symptoms and extraglandular manifestations

  • Sicca is managed by education and environment modification, elimination of contingent offending drugs, artificial tears, secretagogues and treatments for complications

  • Severe and acute systemic manifestations require treatment with glucocorticoids and/or immunosuppressant drugs

  • The role for biologic therapies is controversial as no double-blind randomized controlled trials (RCTs) establishing their efficacy are available

  • Targets for new treatments directed against the immunopathological mechanisms of pSS have been identified

  • Patients who fail to respond to conventional treatment should be invited to participate in ongoing RCTs of novel therapies

Abstract

Primary Sjögren syndrome (pSS) is a progressive autoimmune disease characterized by sicca and systemic manifestations. In this Review, we summarize the available data on topical and systemic medications, according to clinical signs and disease activity, and we describe the ongoing studies using biologic drugs in the treatment of pSS. Expanding knowledge about the epidemiology, classification criteria, systemic activity scoring (ESSDAI) and patient-reported outcomes (ESSPRI) is driving active research. Treatment decisions are based on the evaluation of symptoms and extraglandular manifestations. Symptomatic treatment is usually appropriate, whereas systemic treatment is reserved for systemic manifestations. Sicca is managed by education, environment modification, elimination of contingent offending drugs, artificial tears, secretagogues and treatments for complications. Mild systemic signs such as fatigue are treated by exercise. Pain can require short-term moderate-dose glucocorticoid therapy and, in some cases, disease-modifying drugs. Severe and acute systemic manifestations indicate treatment with glucocorticoids and/or immunosuppressant drugs. The role for biologic agents is promising, but no double-blind randomized controlled trials (RCTs) proving the efficacy of these drugs are available. Targets for new treatments directed against the immunopathological mechanisms of pSS include epithelial cells, T cells, B-cell overactivity, the interferon signature, proinflammatory cytokines, ectopic germinal centre formation, chemokines involved in lymphoid cell homing, and epigenetic modifications.

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Acknowledgements

The authors thank all members of the Brest Diagnosis Primary Sjögren Cohort Study Group: D. Cornec, S. Jousse-Joulin, T. Marhadour, D. Guellec, S. Boisramé-Gastrin, B. Cochener, M. Roguedas-Contios, M. Chastaing, V. Griner-Abraham, F. Couturaud, J. B. Noury, S. Genestet, C. Hanrotel, Y. Renaudineau, P. Marcorelles, S. Costa and Y. Gauvin.

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Authors and Affiliations

  1. EA2216, INSERM ESPRI ERI29, Laboratoire d'Immunothérapie et Pathologies lymphocytaires B, Université de Brest, Labex 'Immunotherapy, Graft, Oncology', Brest Cedex, 29609, France

    Alain Saraux & Valérie Devauchelle-Pensec

  2. Department of Rheumatology, Centre Hospitalier Universitaire de la Cavale Blanche, Brest, 29609, France

    Alain Saraux, Jacques-Olivier Pers & Valérie Devauchelle-Pensec

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  1. Alain Saraux
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All authors researched data for the article and contributed to discussions of content, writing the article, and reviewing and editing the manuscript before submission.

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Correspondence to Alain Saraux.

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Competing interests

J.-O.P. declares that he has received honoraria from Novartis. A.S. declares that he has received funds or honoraria from Abbvie, Bristol Myers Squibb, Chugai, GlaxoSmithKline, MSD, Novartis, Pfizer, Roche and UCB.. V.D.-P. declares that she has received funds or honoraria from Abbvie, Bristol Myers Squibb, Chugai, MSD, Novartis, Roche and UCB.

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Supplementary information S1 (table)

Main immunopathological mechanisms involved in primary Sjögren syndrome (pSS) and potential therapeutic targets (PDF 1515 kb)

Glossary

Focus score

A histopathological score based on the number of mononuclear cell infiltrates containing at least 50 inflammatory cells in a 4mm2 glandular section of the minor salivary gland.

Ocular staining score

(OSS). A score that, similarly to the van Bistjerveld score, is based on a test using a dye (rose bengal or lissamine green) to evaluate damage to conjunctival and corneal epithelial cells. Slight differences exist in the interpretation of these scores: a van Bistjerveld score of 4 is equivalent to an OSS of 5.

Schirmer's test

An objective evaluation of eye dryness using calibrated strips of filter paper placed within the eyelid.

Salivary flow rate

The patient passively drools every minute for 15 min into a 50 ml tube, without or after stimulation. The collected samples are weighed using an analytical balance and expressed in ml per min.

Chronic suppurative sialadenitis

Sudden onset of pain, swelling, indurated, tender major salivary gland (most commonly the parotid gland) with purulence from duct due to bacterial infection.

Sialolithiasis

Postprandial salivary pain and swelling caused by the formation of stones (salts and proteins, predominantly calcium carbonate) in the ductal system; the submandibular gland is most often affected.

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Saraux, A., Pers, JO. & Devauchelle-Pensec, V. Treatment of primary Sjögren syndrome. Nat Rev Rheumatol 12, 456–471 (2016). https://doi.org/10.1038/nrrheum.2016.100

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