Toward a Consensual Evaluation

of Primary Sjogren’s Syndrome

Raphaèle Seror, Philippe Ravaud, Claudio Vitali,

Simon J. Bowman, and Xavier Mariette

Abstract

In primary SjšgrenÕs syndrome (SS), clinical features can be divided into two facets: the benign subjective but disabling manifestations such as dryness, articular and muscular pain, and fatigue, and the systemic manifestations such as synovitis, vasculitis, skin, lung, renal and neurological involvement, or lymphoma. Great efforts have been made to develop valid activity indexes needed to assess the effectiveness of new therapies. First, for evaluation of patientsÕ symptoms: the ProÞle of Fatigue and Discomfort (PROFAD) and Sicca Symptoms Inventory (SSI) then for systemic features: the SS Disease Activity Index (SSDAI) and SjšgrenÕs Systemic Clinical Activity Index (SCAI). The development of these indexes served as bases of an international collaborative project promoted by European League Against Rheumatism (EULAR). Thirty-nine primary SS experts were involved in the development of these two consensus disease activity indexes: the EULAR SjšgrenÕs Syndrome Patients Reported Index (ESSPRI), a patient-administered questionnaire to assess subjective features, and the EULAR SjšgrenÕs Syndrome Disease Activity Index (ESSDAI), a systemic activity index to assess systemic complications. Both indexes have good correlations with existing scores and also global evaluation of disease activity by physician for ESSDAI and by patient for ESSPRI. In addition, ESSDAI had a good sensitivity to change and detects changes more accurately, when compared to other scores. These both indexes are simple and aimed to be used for both clinical trials and clinical practice. They are currently being validated for that purpose.

Keywords

Primary SjšgrenÕs syndrome ¥ Disease activity index ¥ Patient-reported outcome ¥ Outcome assessment ¥ Systemic activity