Epidemiological study of adult-onset Still’s disease using a Japanese administrative database

Abstract

Adult-onset Still's disease (AOSD) is a rare disease, and large epidemiological studies of this disease are limited. Furthermore, it has been difficult to show the incidence and characteristics of severe AOSD complications due to the rarity of this disease. The aim of our study was to describe the demographics of AOSD and the incidence and characteristics of severe complications. Using a large Japanese administrative database, we identified hospitalized patients with AOSD and described the demographics. We also calculated the incidence of severe complications (i.e., macrophage activation syndrome [MAS] and disseminated intravascular coagulation [DIC]) and in-hospital mortality in AOSD patients, and then analyzed the age-controlled difference between men and women. We identified 513 patients with AOSD (mean age: 53.1 years; women 64.1 %). According to the age distribution, there was no distinct peak age. The thirties and the sixties were relatively large age groups. There were 76 patients of AOSD with MAS or DIC observed in this study. The incidence of severe complications was 14.8 %, 95 % CI [11.9, 18.2]. Women were more likely to have severe complications than men after controlling for age (odds ratio: 2.07; [1.14, 3.73]; p = 0.014). AOSD does not predominantly affect young adults in our study population. Elderly AOSD patients can be observed more than before due to global population aging. Severe complications are more likely to occur in women than in men.

Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report and Review of the Literature

“…An in- vestigation from Japan reported that the average age at the onset of AOSD was 38.1±14.3 years old in 1994 (47), in contrast to 46±19 years old in 2010 (44). Another report in 2016 from Japan stated that the mean onset age was 53.1± 19.6 years old (4). About 23% of cases developed it at !…”

“…65 years old and about 16% at ! 75 years old (4). This has been attributed to the marked aging of the population now occurring in Japan (4).…”

“…Macrophage activation syndrome (MAS) and disseminated intravascular coagulation (DIC) are severe complications that adversely affect the prognosis (4). In the treatment of AOSD, glucocorticoid and immunosuppressants, such as cyclosporine (CyA) and methotrexate (MTX), are frequently used.…”

Adult-Onset Still’s Disease

“…1. Supportive measures, which could include platelet transfusion, coagulation factors (or fresh frozen plasma), and fibrinogen (in cryoprecipitate) infusion to control severe bleeding or sometimes heparin if no active bleeding is present [70]. 2.…”

Adult‐onset Still's disease initially thought to be an odontogenic infection: A case report

“…The estimated prevalence of AOSD is 0.14‐0.40 per 100 000 persons . Although the onset is described to be predominant in young adults, a recent study in Japan showed no distinct peak age, with a mean age at onset of 53.1 years, and slight female predominance (64.1%) . The diagnostic criteria are shown in Table .…”