Recurrent Acute Pericarditis as the Initial Presenting Sign in an Adolescent with Primary Sjögren's Syndrome: A Case Report

Abstract

Background: Sjögren’s syndrome (SS) is a chronic autoimmune disease primarily characterized by sicca symptoms, but its presentation in children often differs significantly from that seen in adults. In pediatric cases, classic dryness is uncommon, and the diagnosis is frequently delayed due to atypical manifestations. Cardiac involvement, especially recurrent pericarditis, is sporadic in SS and has not been previously reported as the initial and sole presentation in pediatric patients. Case Presentation: We report the case of a 16-year-old male with no prior medical history who presented with recurrent pericarditis, persistent fever, and systemic symptoms. Extensive evaluation ruled out infectious, neoplastic, and autoinflammatory causes. Although the patient lacked sicca symptoms, a minor salivary gland biopsy revealed focal lymphocytic sialadenitis with a focus score of 1.76, consistent with SS. Serologic testing was notable for isolated anti-Ro52 positivity. The patient was treated with hydroxychloroquine, colchicine, and mycophenolate mofetil, achieving clinical remission during six months of follow-up. Conclusions: This case highlights an atypical presentation of pediatric SS, manifesting solely as recurrent pericarditis without glandular involvement. It underscores the need for heightened clinical suspicion and comprehensive diagnostic evaluation in children with unexplained pericarditis, as early recognition of SS is critical to initiating appropriate immunosuppressive therapy and preventing disease progression. This case underscores the importance of considering autoimmune etiologies in recurrent pericarditis and highlights the need for increased awareness regarding early recognition of primary SS in children. Future research should focus on better defining pediatric manifestations and improving long-term follow-up strategies.