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

Autor: Tatsukata Kawagoe, Michiko Kurosawa, Takeharu Suzuki, Takehito Ishido, Mizuho Ishido, Jun Arimoto, Kentaro Yuta, Takeshi Fukumoto, Shingo Kato, Takahiro Yamane, Yohei Kirino, Nobuhisa Mizuki, Takeshi Kaneko, Mitsuhiro Takeno, Ryuta Mizuki, Etsuko Shibuya, Takahiko Hayashi, Yuki Mizuki, Akira Meguro, Yoshiaki Ishigatsubo, Kaoru Minegishi, Ryusuke Yoshimi, Masaki Takeuchi, Nobuyuki Horita
Rok vydání: 2018
Předmět:
Zdroj: Modern rheumatology. 29(4)
ISSN: 1439-7609
Popis: Background: Clinical data of patients with entro-, vasculo-, and neuro-variant possible Behcet'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.
Databáze: OpenAIRE