Sjogren Syndrome Presenting as Edema and Extensive Pleural Effusion: A Pediatric Case Report

Abstract

Background: To describe a case of pediatric Sjogren syndrome (SS) which initially presented as edema and extensive pleural effusion without sicca symptoms. Case presentation: A 16-year-old female presented to the pediatric nephrology clinic with complaint of bilateral eyelid swelling for approximately 2 months. The laboratory examinations and labial glands biopsy were consistent with SS, for which she was started on a oral prednisone regimen. Despite initial improvement, she re-presented with significantly worsened eyelid swelling and edema of the lower limbs. Abdomen distention and shifting dullness was palpable at this time. Thoracentesis was performed and analysis of hydrothorax indicated exudates. The patient also developed neurological disorder manifesting as static tremor in the course. Intravenous methylprednisolone and immunosuppression therapy was initiated. Reduction of hydrothorax and alleviation of anasarca was observed. She was able to wean off prednisone 7 months later and still remains on mycophenolate mofetil (MMF) therapy after 31 months post diagnosis. Her illness remained stable without any symptoms until now.Conclusions: Sjogren syndrome in the pediatric population may present in diverse ways in the absence of sicca symptoms. We report a case of pediatric SS, presenting with edema and extensive pleural effusion without sicca symptoms, which was successfully treated with immunosuppression. Given the wide spectrum manifestations upon disease onset, it is of great significance to improve our understanding and awareness of this presumably under-diagnosed disease.