Clinical features of early-stage possible Behçet’s disease patients with a variant-type major organ involvement in Japan

Abstract

Background: Clinical data of patients with entro-, vasculo-, and neuro-variant possible Behçet’s disease (BD) based on Japanese criteria has not yet comprehensively reported.

Methods: This ongoing nation-wide registration has been carried out by the Japanese Ministry of Health, Labour and Welfare. The Ministry asked physicians who diagnosed a patient with confirmed or possible BD to register the patient data by filling out a registration form. The Ministry provided us with the dataset after unlinkable anonymization. We analyzed 2003–2014 database generated from the early stage new cases.

Results: Among the 7950 analyzable cases, 694 (8.7%) had variant-type possible BD without satisfying complete/incomplete criteria. Of the 694 patients, 479, 46, and 169 had entero-, vasculo-, and neuro-variant possible BD, respectively. Out of these 694 patients, 35 (5.0%) and 154 (22.2%) satisfied the International Study Group criteria and the International Criteria of BD, respectively. Entero-variant possible patients rarely (1.8%) had ocular lesions. Patients with vasculo-variant possible BD were featured by low genital ulceration risk (6.8%) and frequent positive HLA-B51 (60.0%). Neuro-variant possible BD was featured by high median age at registration (48 year). Vasculo- (69.6%) and neuro-variant (68.6%) BD patients showed clear male dominance. Epididymitis was very rare among variant-type possible BD men.

Conclusion: We analyzed 694 early-stage variant-type possible BD cases. We believe the data from our study will contribute to further international discussion regarding BD diagnostic criteria and clarification of the clinical presentations of the Japanese variant-type possible BD patients.

Keywords:

• Behçet syndrome
• gastrointestinal tract
• blood vessels
• central nervous system

Acknowledgement

We would like to thank the Japanese Ministry of Health, Labour and Welfare for providing the nationwide BD survey database. This work was partly supported by the Health and Labour Sciences Research Grants (Research on Intractable Diseases) from the MHLW of Japan.

Conflict of interest

None.