Childhood-onset Sjögren disease : clinical phenotype compared with adult-onset disease, disease course and treatment patterns in a nationwide Italian multicentre pediatric cohort
Abstract
Childhood-onset Sjögren disease (cSjD) is a rare autoimmune disease with an incompletely characterized phenotype, limited longitudinal data, no validated classification criteria, and limited evidence to guide treatment. To compare the clinical and serological phenotype of childhood-onset and adult-onset Sjögren disease (SjD) and to describe disease course of the pediatric cohort. This cross-sectional study included children with cSjD fulfilling the SjD 2016 ACR/EULAR criteria recruited from 22 Italian pediatric rheumatology centres and adult-onset SjD patients from a tertiary referral centre. Demographic, clinical, laboratory, ESSDAI, clinESSDAI, ESSPRI, and treatment were compared. Longitudinal pediatric data were analysed descriptively, and factors associated with systemic treatment initiation were explored. The final cohorts included 102 cSjD and 140 adult patients. Children more frequently presented with recurrent parotid enlargement (44% vs. 13%, p < 0.0001), cutaneous involvement (25% vs. 8%, p < 0.0001), lymphadenopathy (24% vs. 9%, p = 0.001), arthritis (17.7% vs. 6.4%, p = 0.006), and fever (12% vs. 3%, p = 0.006), whereas sicca symptoms were significantly less common (xerostomia 45% vs. 94%; xerophthalmia 36% vs. 86%; both p < 0.0001). cSjD showed higher ESSDAI (median 5.5 vs. 1.0, p < 0.0001), higher serum IgG, but lower anti-SSA/anti-SSB positivity. During a 26.4-months median follow-up, 87.5% initiated systemic treatment. In exploratory analyses, higher baseline ESR was independently associated with a shorter time to systemic treatment initiation. Compared with adult-onset SjD, cSjD showed more frequent systemic inflammatory involvement and less prominent sicca manifestations. Pediatric-specific classification criteria, outcome measures, and prospective studies to optimise management and improve long-term outcomes are still crucial unmet needs.
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Authors: Flavia Riccio, Silvana Ancona, Simona Campus, Achille Marino, Gabriele Simonini, Francesco Baldo, Edoardo Marrani, Valentina Ibba, Angela Mauro, Alessandra Meneghel, Donato Rigante, Emanuela Del Giudice, Serena Pastore, Valeria Manfrè, Adele Civino, Carlotta Covizzi, Roberta Naddei, Andrea Uva, Roberta Loconte, Luciana Breda, Michela Cappella, Sofia D’Elios, Emma Longoni, Francesca Alessi, Maria Francesca Gicchino, Enrico Tirri, Sara Pessano, Angela Pistorio, Marco Gattorno, Francesco Ciccia, Saviana Gandolfo, Clara Malattia
Institutions: University of Padua, Istituti di Ricovero e Cura a Carattere Scientifico, Santobono Children's Hospital, University of Chieti-Pescara, Sapienza University of Rome, University of Campania "Luigi Vanvitelli", University of Naples Federico II, University of Milan, University of Udine, Meyer Children's Hospital, Ospedale Vito Fazzi, Azienda Ospedaliera Citta' della Salute e della Scienza di Torino, University of Genoa, University of Campania "Luigi Vanvitelli", University of Ferrara, Università Cattolica del Sacro Cuore, Agostino Gemelli University Polyclinic, Istituto Giannina Gaslini, Ospedale Regina Margherita, Ospedale Santa Chiara, ASST Fatebenefratelli Sacco, Azienda Socio Sanitaria Territoriale Lariana, Azienda Sanitaria Unità Locale di Reggio Emilia, IRCCS Materno Infantile Burlo Garofolo, Azienda sanitaria universitaria Friuli Centrale, Ospedale Pediatrico Giovanni XXIII, Ospedale Microcitemico