TY - JOUR
T1 - Is early-onset primary Sjögren's syndrome a worse prognosis form of the disease?
AU - Anquetil, Céline
AU - Hachulla, Eric
AU - MacHuron, François
AU - Mariette, Xavier
AU - Le Guern, Véronique
AU - Vittecoq, Olivier
AU - Dernis, Emmanuelle
AU - Larroche, Claire
AU - Dubost, Jean Jacques
AU - Perdriger, Aleth
AU - Devauchelle-Pensec, Valérie
AU - Fauchais, Anne Laure
AU - Morel, Jacques
AU - Dieudé, Philippe
AU - Rist, Stéphanie
AU - Sene, Damien
AU - Gottenberg, Jacques Eric
AU - Hatron, Pierre Yves
N1 - Publisher Copyright:
© The Author(s) 2018.
PY - 2019/7/1
Y1 - 2019/7/1
N2 - 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.
AB - 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.
KW - Disease activity
KW - Epidemiology
KW - Sjögren's syndrome
UR - http://www.scopus.com/inward/record.url?scp=85068483026&partnerID=8YFLogxK
U2 - 10.1093/rheumatology/key392
DO - 10.1093/rheumatology/key392
M3 - Article
C2 - 30561748
AN - SCOPUS:85068483026
SN - 1462-0324
VL - 58
SP - 1163
EP - 1167
JO - Rheumatology
JF - Rheumatology
IS - 7
ER -