Funding body: FOREUM (Foundation for Research in Rheumatology)
Dates: 2018-2021
Coordinator: W-F Ng, Newcastle University
Consortium: 8 partners, 4 countries
Budget: €600,000
Project website

Primary Sjögren’s syndrome (PSS) is a chronic complex immune-mediated rheumatic disease with no effective treatment to date. PSS affects 0.05-0.1% of the adults. A key barrier to therapeutic development is the marked heterogeneity in clinical manifestations and pathobiological profiles among PSS patients.

This FOREUM project aims at providing a deep understanding of the clinical and pathobiological characteristics of four primary Sjögren's syndrome (pSS) subtypes based on patient-reported levels of dryness, pain, fatigue, anxiety and depression (scored using the EULAR Sjögren’s syndrome patient reported index (ESSPRI) and Hospital anxiety and depression scale). The 4 subtypes are termed “Low Symptom Burden (LSB)”, “Dryness Dominant with Fatigue (DDF)”, “Pain Dominant with Fatigue (PDF)” and “High Symptom Burden (HSB)”.

The specific objectives are:

1- to understand the natural history and health-economic burden of the pSS subtypes through collection of longitudinal outcome data from eight European cohorts;
2- to further characterise the underpinning pathotypes of these four pSS subtypes through additional analyses of samples from blood and biopsy tissues from target organs (salivary glands);
3- to explore whether the pSS subtypes respond differently to therapies by re-analysing data from published clinical trials stratifying according to these pSS subtypes.

This study will provide a deep understanding of the clinical and pathobiological characteristics of the four pSS subtypes. This study will also establish a rich bio-resource (dataset & biobank) of these pSS subtypes for biomarker discovery, novel therapy development and appropriate clinical endpoints for each pSS subtype in future therapeutic trials.

LBAI is involved in patient’s recruitment (V Devauchelle-Pensec and A Saraux).