Clinical practice guidance for Sjögren’s syndrome in pediatric patients (2018) – summarized and updated

Abstract

There are a considerable number of pediatric patients with Sjögren’s syndrome (SS); however, SS is generally considered rare among children. Pediatric patients with SS report fewer sicca symptoms; therefore, many are under-diagnosed and cannot access appropriate medical management. Therefore, we propose a newly developed guidance for the diagnosis, treatment, and management of pediatric SS, including epidemiology, clinical features, and diagnostic examination methodology. The aim of this guidance was to standardize the medical care of pediatric SS in Japan, and we published the Japanese version by YODOSHA in 2018. This article is the English version, which is summarized and updated. This guidance will need to be revised in the near future as additional clinical data become available.

Keywords:

• Algorithm
• diagnostic guidance
• early stage
• follow-up
• management

Acknowledgement

The authors thank Dr. Hisanori Umehara, Dr. Ichiro Saito, Dr. Yasunori Sato, Dr. Etsuko Takamura, and Dr. Toshio Yoshihara for their helpful comments in developing the diagnostic guidance.

Conflict of interest

Masaaki Mori: Tokyo Medical and Dental University (TMDU) received unrestricted research grants for the Department of Lifetime Clinical Immunology from AbbVie GK, Ayumi Pharmaceutical Corporation, Chugai Pharmaceutical Co., Ltd., CSL Behring K.K., Japan Blood Products Organization, Nippon Kayaku Co., Ltd., UCB Japan Co., Ltd., and Asahikasei Pharmaceutical Corporation. All other authors have declared no conflict of interest.

Additional information

Funding

Research on rare and intractable diseases, Health and Labor Sciences Research Grants for ‘Study about the standardization of diagnostic criteria and disease severity classification and the development of evidence-based treatment guidelines for pediatric rheumatic diseases such as mainly juvenile idiopathic arthritis’ (H27-nanchitou(nan)-ippan-029).