Estimated Prevalence and Incidence of Adult Still's Disease: Findings by a Nationwide Epidemiological Survey in Japan

Wakai, Kenji; Ohta, Akio; Tamakoshi, Akiko; Ohno, Yoshiyuki; Kawamura, Takashi; Aoki, Rie; Kojima, Masayo; Lin, Yingsong; Hashimoto, Shuji; Inaba, Yutaka; Minowa, Masumi; Aizawa, Shigeo; Ichikawa, Yōichi; Miyasaka, Nobuyuki

doi:10.2188/jea.7.221

Cited by 147 publications

(91 citation statements)

References 9 publications

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“…In Japan, the prevalence among men and women over the age of 16 years has been estimated to be 0.73/100,000 and 1.47/100,000, while the crude incidence rates are 0.22 and 0.34, respectively [30]. The yearly incidence in the Caucasian population seems to be lower [31].…”

Section: Discussionmentioning

confidence: 99%

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

Priori

Colafrancesco

Gattamelata

et al. 2010

Autoimmun Highlights

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Purpose The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. Methods The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature. Results The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm 3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). Conclusion The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

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Section: Discussionmentioning

confidence: 99%

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

Priori

Colafrancesco

Gattamelata

et al. 2010

Autoimmun Highlights

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“…The estimation was based on the assumption that the responses of the departments were independent of the frequency of patients [8,10]. Formulas used to estimate the total number of patients, and the 95% confidence intervals are described below.…”

Section: Estimation and Statistical Analysismentioning

confidence: 99%

Nationwide survey on the epidemiology of syringomyelia in Japan

Sakushima

Tsuboi

Yabe

et al. 2012

Journal of the Neurological Sciences

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“…In a Dutch study of 45 patients, women were 60% and the median age of presentation was 25 years (range 16 to 65 years), with 27% of the patients showing onset of symptoms after the age of 35 [8]. An epidemiologic analysis from Japan evaluated the incidence amongst men at 0.22 per 100,000 inhabitants and at 0.34 per 100,000 amongst women [9].…”

Section: Epidemiologymentioning

confidence: 99%