An Early View of Iran Primary Sjogren Syndrome Registry (Guilan Province Pilot Phase), an Emerging Effort for a National Registry Establishment.

Background: The Sjogren syndrome (SS) is the least well-known rheumatic condition. We aim to gradually resolve it by standardizing the process of SS care in our country, at first for primary Sjogren syndrome cases, through developing Iran Primary Sjogren Syndrome Registry (IRAPSS) which will create a representative and comparable data bank.

Methods: Guilan Primary Sjogren Syndrome Registry (GUIRAPSS) is a pilot phase of IRAPSS, which is a prospective cohort. Care of its patients is done based on EULAR Sjögren's syndrome disease activity index (ESSDAI) recommendations. Other IRAPSS outcome measurements are EULAR Sjogren syndrome patient reported index (ESSPRI) and Sjögren's syndrome disease damage index (SSDDI) for detecting disease damage.

Results: Female-male ratio was 17.5. The age at expert diagnosis was 46.97 ± 11.93 years old. The most common comorbidity was hypothyroidism (28.38%), followed by musculoskeletal conditions (27.02%). 28.38% had fatigue. Active disease existed based on the clinical form of ESSDAI and ESSDAI, 31.8% and 81.8% respectively. The most involved organs during disease activity based on ESSDAI were biological 68.12% following pulmonary (12.16%) and respiratory (10.81%) system. Hydroxychloroquine was the most prescribed drug (72.97%), followed by prednisolone (28.38%).

Conclusion: Disease registries provide an ideal opportunity for gathering standardized and comparable data which provides needed items for creating, updating, or adapting pSS classification criteria/diagnosis, outcome measurements and or treatment guidelines.