Céline Anquetil1, Eric Hachulla1, François Machuron2, Xavier Mariette3, Véronique Le Guern4, Olivier Vittecoq5, Emmanuelle Dernis6, Claire Larroche7, Jean-Jacques Dubost8, Aleth Perdriger9, Valérie Devauchelle-Pensec10, Anne-Laure Fauchais11, Jacques Morel12, Philippe Dieudé13, Stéphanie Rist14, Damien Sene15, Jacques-Eric Gottenberg16, Pierre-Yves Hatron1. 1. Service de médecine interne, CHU Claude Huriez, Lille Cedex, France. 2. Department of Biostatistics, Univ. Lille, CHU Lille, EA 2694 - Santé Publique : Épidémiologie et Qualité des Soins, Lille, France. 3. Service de rhumatologie, Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Sud, Le Kremlin-Bicêtre, France. 4. Centre de référence des maladies auto-immunes rares, CHU Cochin APHP, Paris, France. 5. Service de rhumatologie, CHU, Rouen, France. 6. Service de rhumatologie, Centre hospitalier Le Mans, Le Mans Cedex, France. 7. Service de médecine interne, Hôpital Avicenne APHP, Bobigny Cedex, France. 8. Service de rhumatologie, CHU Gabriel Montpied, Clermont-Ferrand Cedex, France. 9. Service de rhumatologie, CHU, Rennes, France. 10. Service de rhumatologie, CHU Cavale Blanche, INSERM 1227, Brest Cedex, France. 11. Service de médecine interne, CHU, Limoges, France. 12. Département de rhumatologie, CHU de Montpellier, Université de Montpellier, Montpellier, France. 13. Service de rhumatologie, Hôpital Bichat AP-HP, Paris, France. 14. Service de rhumatologie, CHU, Orléans, France. 15. Département de médecine interne, Hôpital Lariboisière APHP, Paris, France. 16. Service de rhumatologie, CHU Hautepierre, Strasbourg, France.
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.
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.
Authors: Rachel L Randell; Sara M Stern; Heather Van Mater; Laura E Schanberg; Scott M Lieberman; Matthew L Basiaga Journal: Pediatr Rheumatol Online J Date: 2022-09-05 Impact factor: 3.413
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Authors: Lin Wei; Xin Zhifei; Ning Xiaoran; Liu Meilu; Li Yang; Liu Yixuan; Ren Xiuying; Su Yashuang; Cao Jingjing; Guo Shaoying; Yang Liu; Sun Lijun; Zhang Fengxiao; Zhang Wen Journal: Rheumatology (Oxford) Date: 2022-02-02 Impact factor: 7.580