The British Society for Rheumatology Guideline for the Management of Adults With Primary Sjögren's Syndrome

Elizabeth J. Price; Saaeha Rauz; Anwar R. Tappuni; Nurhan Sutcliffe; Katie L. Hackett; Francesca Barone; Guido Granata; Wan-Fai Ng; Benjamin A. Fisher; Michele Bombardieri; Elisa Astorri; Ben Empson; Genevieve Larkin; Bridget Crampton; Simon J. Bowman


Rheumatology. 2017;56(10):1643-1647. 

In This Article

Executive Summary


Primary SS (pSS) is a classic, immune-mediated, condition[1] typically presenting in women in their fifth or sixth decade, although up to 10% of cases occur in men. The prevalence in women in the UK is 0.1–0.4%.[2] Patients characteristically present with dryness of the eyes and mouth but systemic features are common and B-cell lymphoma affects 5–10%.[3,4]

Objective of Guideline

This document aims to provide a pragmatic, practical guideline for the management of adults with pSS. The Full Guideline is available at Rheumatology Online.

Target Audience

The target audience includes rheumatologists, general physicians, general practitioners, specialist nurses and other specialists (e.g. ophthalmologists, dental practitioners and ENT specialists).

Areas not Covered

This guideline does not cover the detailed management of patients with secondary Sjögren's or patients with lymphoma, who should be managed in conjunction with oncologists and haematologists. The management of children is not specifically covered although is similar to that for adults with special emphasis on good dental care and hygiene.

Rigor of Development and Limitations

A search was undertaken for all relevant evidence from 1990 to January 2016. The level of evidence (LOE) was graded from Ia through to IV and A through to D. See Appendix 1 for full details of search criteria and LOE definitions.

A strength of agreement (SOA) was calculated for each recommendation and the results expressed as an SOA score (0–10) with percentage of respondents scoring the recommendation ⩾7 in parentheses. Recommendations were only included where the mean SOA was ⩾7 and ⩾75% respondents scored ⩾7.