Sjögren's syndrome in children: about 15 cases in Guinea Conakry

Eur J Med Res. 2021 Jul 2;26(1):66. doi: 10.1186/s40001-021-00534-6.

Abstract

Objectives: Sjögren's syndrome is rare in children and most often secondary. It frequently affects girls and is characterized by dry eye syndrome, mouth and sometimes systemic involvement. Its diagnosis is difficult to establish in children. We report a series of 15 cases of Sjögren's syndrome in order to clarify the peculiarities of this condition in children.

Patients and methods: This retrospective study was carried out over a 2-year period focused on children under 16 years of age who had been followed for Sjögren's syndrome in the rheumatology and pediatric departments. Patient data were collected and then analyzed by STATA/SE version 11.2 software. Anonymity and respect for ethical rules were the norm. There was no connection between the patients and the researchers.

Description of cases: The mean age of the patients was 11 years with extremes of 5-15 years. History reveals that a dry mouth was found in more than half of the cases, or in 10 (66.7%) patients. Clinical examination found oral ulceration and periodontitis in equal proportions, 6 (40%). The immunological workup and the biopsy of the accessory salivary glands served as diagnostic evidence in the 15 patients according to the US-European criteria of 2002.

Conclusion: Sjögren's syndrome is a rare entity in pediatrics. Its diagnosis is difficult to establish in pediatrics and its severity is linked to the occurrence of late visceral and lymphomatous sicca syndrome. Rapid diagnosis and initiation of a synthetic antimalarial (hydroxychloroquine) increases the hope of a cure.

Keywords: Children; Conakry; Guinea; Sjögren’s syndrome.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Guinea / epidemiology
  • Humans
  • Male
  • Morbidity / trends
  • Retrospective Studies
  • Salivary Glands / diagnostic imaging*
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / epidemiology*