Background Although primary Sjögren’s syndrome (pSS) is often a benign condition some patients develop systemic features. Identifying independent risk factors for pSS different clinical phenotypes would provide useful insights into patients’ assessment.
Objectives to describe the clinical presentation of pSS in a large cohort of patients and to identify independent risk factors for severe disease manifestations.
Methods Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent risk factors for disease manifestations were identified by logistic regression.
Results The cohort included 1115 (1067 F:48M) pSS patients with a mean age of 51.6±13.8 yrs at diagnosis and 57.5±13.7 yrs at inclusion in the study (mean follow-up=5.8±6.5 yrs). Anti-Ro/SSA and anti-La SSB were detected in the 68% and 37% of the cases. Xerostomia (92%), xerophtalmia (94%) and articular involvement (61%) were the most commonly detected clinical manifestations. Salivary gland enlargement was detected in 346/1115 (31%) pSS patients while 475/1115 (42%) developed systemic extra-glandular involvement. Moderate to severe leukopenia (28%), peripheral arthritis (11%) and sensory-motor neuropathy (1.8%) were the most severe disease extra-glandular manifestations requiring immunosuppressive drugs. Independent risk factors for leukopenia were Raynaud’s phenomenon (RP) (p=0.008), low C3 (p=0.001), hypergammaglobulinemia (p=0.0006) and cryoglobulinemia (p=0.01). Arthritis was associated to RP (p=0.005) while sensory-motor neuropathy to purpura (p<0.0001), low C4 (p<0.0001) and cryoglobulinemia (p<0.0001). From the analysis, low C3/C4, hypergammaglobulinemia and cryoglobulinemia emerged as prognostic adverse laboratory abnormalities. Table 1 summarises patients’ clinical presentation according to the number of the laboratory abnormalities detected (“group A” = no abnormalities, “group B” =1 and “group C” ≥2 abnormalities).
Conclusions This study described the presentation of pSS in a large cohort of patients allowing us to identify independent risk factors which might help to predict the signs and symptoms of the disease. Patients with laboratory signs of B-cell hyperactivation should be more closely monitored since they may be at higher risk of severe extra-glandular involvement.
Disclosure of Interest None Declared