AB1040 PEDIATRIC SJöGREN SYNDROME: A MONOCENTER CASE SERIES
Authors: micol romano, Gabriella Maioli, Angela Flavia Luppino, Maurizio Gattinara, Valeria Gerloni
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Sjögren syndrome (SS) is a chronic autoimmune disorder characterized by inflammation of the lacrimal and salivary glands leading to oral and ocular dryness. Childhood SS is rare and poorly defined and underdiagnosed owing to the lack of child-specific diagnostic or classification criteria [1]. <h3>Objectives</h3> The purpose of this study is to describe five cases with pediatric SS in order to better clarify the characteristics of the disease in the childhood. <h3>Methods</h3> We retrospectively reviewed medical records of patients with pediatric SS referring to our center (ASST-PINI-CTO, University of Milan) from November 2008 to December 2018. No adequate childhood SS-specific criteria exist. Therefore we select our pts according to a combination of clinical, serological, histopatological findings. For the assessment of systemic disease activity we applied ESSDAI (EULAR Sjögren's syndrome disease activity index). We calculated ESSDAI at diagnosis, at the peak disease activity and at the last follow up visit. <h3>Results</h3> We collected data on 5 pts (4 females). The mean of age at onset is 8.4 yrs (range 4.8-11.2). The mean of age at diagnosis is 10.8 (range 6.5-13.3). The follow up period varied from 1.2 to 9.3 yrs (mean 6.3). The most common manifestations were parotid/salivary glands swelling (5/5 pts) and articular involvement (5/5 pts) with arthritis in 2 pts and arthralgia in the others. Nobody of our pts presented dryness of eyes. Xerostomia was found in 2/5 pts. Fever and fatigue occurred in 2/5 and 3/5 pts respectively. We also recorded 2 cases of abdominal pain, 1 case of glomerular proteinuria, 2 cases of purpura and 1 case of erythema nodosum. ANA, anti-SSA, anti-SSB, RF, elevation of ERS and hypergammaglobulinemia (1.6 – 8.04 g/dl) were present in all cases. Minor salivary gland biopsy was performed in 4 pts resulting in histological evidence of focal lymphocytic sialadenitis (one of them showed chronic sialadenitis with a focus score &lt;1/4mm2). Ultrasound studies of salivary glands were positive in 5/5 cases. Mean ESSDAI scores at diagnosis, peak activity and the last follow up visit were 8 (range 5-11), 13.2 (9-20), 4.8 (0-9). The most frequently involved domains at the peak activity were biological (5/5), parotid/salivary glands swelling (5/5) and articular (5/5). No major complications have been observed. With regard to treatment, all patient received corticosteroids and DMARDs (5/5 hydroxychloroquine, 3/5 methotrexate, 1/5 azathioprine, 1/5 leflunomide); one patient needed plasmapheresis and immunosuppressive therapy with cyclophosphamide. Biological therapy was administered to 2 patients: 1 received belimumab and then rituximab, while the other patient received rituximab. <h3>Conclusion</h3> Xerostomia and keratoconjunctivitis sicca are not a common features in children, on the other hand juvenile SS has higher incidence of recurrent parotid swellings than adults [2, 3]. All pts had laboratory abnormalities. The long-term prognosis of pediatric SS is unknown, in our series only 2 patients were inactive (ESSDAI score &lt;5, according to the definition by EULAR) at last evaluation, 3 were moderately active with a minimal clinically important improvement (MCII) observed in all of them [4]. <h3>References</h3> [1] Lieberman SM. Curr Opin Rheumatol, 2013. [2] Kobayashi I et al, Modern Rheumatology, 2018. [3] Cimaz Ret al, Eur J Pediatr, 2003 [4] Seror Ret al, RMD Open, 2015. <h3>Disclosure of Interests</h3> None declared