Is early-onset primary Sjögren's syndrome a worse prognosis form of the disease?. (17th December 2018)

• Record Type: Journal Article
• Authors: Anquetil, Céline; Hachulla, Eric; Machuron, François; Mariette, Xavier; Le Guern, Véronique; Vittecoq, Olivier; Dernis, Emmanuelle; Larroche, Claire; Dubost, Jean-Jacques; Perdriger, Aleth; Devauchelle-Pensec, Valérie; Fauchais, Anne-Laure; Morel, Jacques; Dieudé, Philippe; Rist, Stéphanie; Sene, Damien; Gottenberg, Jacques-Eric; Hatron, Pierre-Yves
• Is Part Of: Rheumatology. Volume 58:Number 7(2019)
• Page Start: 1163
• Page End: 1167
• Publication Date: 2018-12-17
• Subjects: Sjögren's syndrome -- disease activity -- epidemiology; Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/key392
• Languages: English
• ISSNs: 1462-0324

Abstract

Abstract: Objectives: Onset of primary SS is usually between 40 and 60 years of age, with severe systemic complications in 15% of cases. We sought to determine whether early-onset disease is related to a specific phenotype and if it is predictive of a poor outcome. Methods: Biological and clinical data from 393 patients recruited in the ASSESS cohort, a French multicentre prospective cohort, were compared according to age at diagnosis. Results: Fifty-five patients had early-onset disease, defined as age ⩽35 years at diagnosis, and presented a significantly higher frequency of salivary gland enlargement (47.2% vs 33.3%, P = 0.045), adenopathy (25.5% vs 11.8%, P = 0.006), purpura (23.6% vs 9.2%, P = 0.002) and renal involvement (16.4% vs 4.4%, P = 0.003). They had a higher frequency of hypergammaglobulinaemia (60.8% vs 26.6%, P < 0.001), RF positivity (41.5% vs 20.2%, P < 0.001), low C3 level (18.9% vs 9.1%, P = 0.032), low C4 level (54.7% vs 40.2%, P = 0.048) and autoantibodies [84.6% with anti-SSA vs 54.4% ( P < 0.001) and 57.7% with anti-SSB vs 29.7% ( P < 0.001)]. The change in ESSDAI scores between baseline and the 5-year follow-up was significantly different ( P = 0.005) with a trend for worsening in the early-onset group (0.72, P = 0.27) and a significant improvement in the later onset group (−1.27, P < 0.0001). Conclusion: Early-onset primary SS is associated with a specific phenotype defined by clinical and biological features known to be predictive factors of severe systemic disease. Interestingly, we showed a different evolution of the ESSDAI score depending on the age at disease onset, patients with early-onset disease tending to worsen over time.