Revised Japanese criteria for Sjögren’s syndrome (1999): availability and validity

Abstract

The Japanese criteria for diagnosing Sjögren's syndrome (SS) were revised in 1999, and consist of four major areas: histopathology, oral examination, ocular examination, and serological examination. A diagnosis of SS can be made when the patient meets at least two of these four criteria. This report describes how the revised Japanese criteria were established. After the publication of the revised Japanese criteria (1999), a research study which focused on evaluating its availability and validity was carried out in 2001 using funds from Grant-in-Aids for Scientific Research supported by the Japan Society for the Promotion of Science. The availability of the revised criteria was investigated by a questionnaire study through the Japanese Medical Society for Sjögren's Syndrome, and the use of the revised criteria for diagnosing SS in these medical facilities was found to be 76%. To evaluate the validity of the revised criteria, the records of 900 patients, including SS patients and non-SS controls, from 54 clinical centers were registered and analyzed to calculate the accuracy of the criteria. The revised Japanese criteria were found to have 96.0% sensitivity, 90.5% specificity, and 94.5% accuracy for diagnosing SS.

Clinical features of 405 Japanese patients with systemic sclerosis

“…Systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) were diagnosed according to the 1982 and 1987 criteria of the American Rheumatism Association (now American College of Rheumatology), respectively [16,17]. Sjögren's syndrome was diagnosed according to the 1999 Japanese Ministry of Health and Welfare's diagnostic criteria for Sjögren's syndrome [18]. Myositis was defined as inflammation of skeletal muscle associated with a serum creatine kinase level above the normal institutional range.…”

The immunobiology and clinical characteristics of IgG4 related diseases

“…A total of 70 SS cases and 70 MD cases were analyzed. The diagnosis of SS was made according to the revised Japanese criteria with positivity of antieSSeA antibody as a minimum requirement [7] and all cases were corresponding to primary SS. The diagnosis of MD was made according to the diagnostic criteria for IgG4-related MD determined by the Japanese Society for SS [8].…”

Efficacy and safety of orally administered pilocarpine hydrochloride for patients with juvenile-onset Sjögren’s syndrome

“…This study was conducted in accordance with the Declaration of Helsinki and the Good Clinical Practice (GCP) guidelines. To be included in the study, patients had to fulfill the revised Japanese SS diagnostic criteria (1999) [4]. Patients' age at entry ranged from 6 to 17 years, and the age at onset was younger than 16 years.…”