Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet's disease in Japan.

BACKGROUND: We hypothesized that Behçet's disease (BD) consists of several clinical subtypes with different severity, resulting in heterogeneity of the disease. Here, we conducted a study to identify clinical clusters of BD. METHODS: A total of 657 patients registered in the Yokohama City University (YCU) regional BD registry between 1990 and 2018, as well as 6754 patients who were initially registered in the Japanese Ministry of Health, Labour and Welfare (MHLW) database between 2003 and 2014, were investigated. The YCU registry data regarding the clinical manifestations of BD, human leukocyte antigen (HLA) status, treatments, and hospitalizations were analyzed first, followed by similar analyses of the MHLW for validation. A hierarchical cluster analysis was independently performed in both patient groups. RESULTS: A hierarchical cluster analysis determined five independent clinical clusters in the YCU cohort. Individual counterparts of the YCU clusters were confirmed in the MHLW registry. Recent phenotypical evolutions of BD in Japan, such as increased gastrointestinal (GI) involvement, reduced complete type according to the Japan Criteria, and reduced HLA-B51 positivity were associated with chronologically changing proportions of the clinical clusters. CONCLUSIONS: In this study, we identified independent clinical clusters among BD patients in Japan and found that the proportion of each cluster varied over time. We propose five independent clusters namely "mucocutaneous", "mucocutaneous with arthritis", "neuro", "GI", and "eye."

Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet's disease: a retrospective observational study.

BACKGROUND: It has been suggested that the phenotypes of Behçet's disease (BD) in Japan are changing. To ask whether the evolution of BD holds true in recent-onset cases in Japan, we performed a retrospective study. METHODS: We reviewed the records of 578 patients with BD who met the 1987 revised diagnostic criteria of the Behçet's disease research committee of Japan. The patients were divided into three groups based on the date of disease onset. We compared the demography, clinical features, and treatments among them with or without adjustment for the observation period. Patients having oral ulcers, genital ulcers, regional skin involvement, and uveitis are categorized as having complete-type BD, and the associated factors were determined by univariate and multivariate logistic regression analyses. RESULTS: Male patients had a higher propensity for uveitis and central nervous system (CNS) involvement, whereas female patients had higher rates of genital ulcers and arthritis. We found a significant trend in reduction of complete-type, genital ulcer, HLA-B51 carriers, and increment of gastrointestinal BD over time. Multiple regression analysis identified HLA-B51 positivity, earlier date of disease onset, and younger age of onset as independently associated with complete-type BD. Although treatments had been also chronologically changed, the causative relationship between therapeutic agents and phenotypical changes was not determined from the study. CONCLUSION: The present study revealed that phenotypical evolution was characterized by decreased incidence of the complete type and increment of gastrointestinal involvement in Japanese patients with BD during the last 30 years.

[Effect of pneumonia on clinical course and prognosis after hip fracture].

The records of 38 elderly patients with hip fracture admitted to our hospital between January and December 2002 were retrospectively reviewed to determine the incidence and outcomes of medical complications. The mean age was 84.5 +/- 6.83 years old, 32 women and 6 men. 27 patients (71%) suffered from dementia. Of the 38 patients, 33 (86.8%) had one and more underlying diseases: hypertension 29, cerebrovascular episode 7, congestive heart failure 5, diabetes mellitus 4, gastric ulcer or chronic gastritis 3, ischemic heart disease 4, depression 2. Three patients had a past history of hip fracture. Fourteen patients (37%) developed medical complications after hip fracture, most frequently pneumonia (64%). Other complications were dizziness, nausea, congestive heart failure, choledocholithiasis, and GI tract bleeding. Eight patients who suffered pneumonia cancelled elective surgery. Severity of pneumonia was mild in 2, moderate in 5, and severe in 2. Both cases with severe pneumonia died in hospital. Patients with pneumonia (pneumonia group) were significantly older and had more severe dementia than patients without pneumonia (non-pneumonia group). Although there were no significant differences in physical ability between the two groups before admission, physical ability on discharge was lower in the pneumonia group. The pneumonia group had a significantly longer mean hospital stay than the non-pneumonia group. Our results suggest that the prevention of pneumonia is necessary to improve the outcome of hip fracture.