Characteristics and treatment of Sjögren’s syndrome in childhood

Abstract

　Sjogren’s syndrome is a systemic autoimmune disease with elevated autoantibody titer, centered on exocrine glanditis in the lacrimal and salivary glands. At diagnosis, adult cases often show dry symptom of exorine glands. On the other hand, frequency of dryness of exorine glands in childhood with Sjogren’s syndrome is low. This is considered to be due to the short term of exocrine glanditis present in children compared with adults. Children with poor dryness often visit medical institutions with symptoms such as fever, repetitive parotitis, arthragia/arthritis, rash, general fatigue. It is estimated that many cases are not diagnosed with Sjogren’s syndrome, because typical dry symptoms are less frequent. For diagnosis, it is necessary for serologic evaluation and evaluation of exocrine glands. Secretion of tears and/or saliva is not likely to decrease, and labial small salivary gland biopsy (lip biopsy) is important for diagnosis. It seems necessary to conduct a long-term observation, such as whether we can prevent dryness by initiating immunosuppressive therapy before function decline of exocrine glands. Regarding treatment of extra-glandular symptoms, treat each case and organ dysfunction according to its degree. Steroids and/or Immunosuppressive agents may require aggressive treatment in some cases. Compare the comparative examples. Outlines the characteristics and treatment of childhood SS.