Racial/Ethnic variations in morbidity and mortality in Adult Onset Still's Disease: An analysis of national dataset

Mehta, Bella; Ibrahim, Said A.; Briggs, William T.; Efthimiou, Petros

doi:10.1016/j.semarthrit.2019.04.004

Cited by 43 publications

(46 citation statements)

References 23 publications

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“…In the sJIA subgroup males and females were equally represented, whereas AOSD sample was skewed toward females, who composed 65.8% of the population. This is in accordance with previous studies reporting almost exactly the same percentage (Sakata et al, 2016; Mehta et al, 2019).…”

Section: Discussionsupporting

confidence: 94%

Anakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

et al. 2019

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Background and Objective: Only a few studies have reported long-term efficacy of interleukin (IL)-1 inhibition in systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still disease (AOSD). Herein we report on the effectiveness of anakinra (ANA), expressed in terms of drug retention rate (DRR), and evaluate the predictive factors of drug survival in a cohort of patients with sJIA and AOSD. Patients and Methods: This is a multicenter study reviewing retrospectively the medical records from 61 patients with sJIA and 76 with AOSD, all treated with ANA in 25 Italian tertiary referral centers. Results: The cumulative retention rate of ANA at 12-, 24-, 48-, and 60-month of follow-up was 74.3%, 62.9%, 49.4%, and 49.4%, respectively, without any significant differences between sJIA and AOSD patients ( p = 0.164), and between patients treated in monotherapy compared with the subgroup coadministered with conventional disease-modifying antirheumatic drugs (cDMARDs) ( p = 0.473). On the other hand, a significant difference in DRR was found between biologic-naïve patients and those previously treated with biotechnologic drugs ( p = 0.009), which persisted even after adjustment for pathology ( p = 0.013). In the regression analysis, patients experiencing adverse events (AEs) {hazards ratio (HR) = 3.029 [confidence interval (CI) 1.750–5.242], p < 0.0001} and those previously treated with other biologic agents [HR = 1.818 (CI 1.007–3.282), p = 0.047] were associated with a higher HR of ANA discontinuation. The median treatment delay was significantly higher among patients discontinuing ANA ( p < 0.0001). Significant corticosteroid-sparing ( p = 0.033) and cDMARD-sparing effects ( p < 0.0001) were also recorded. Less than one-third of our cohort developed AEs, and 85% were deemed mild in nature, with 70% of them involving the skin. Conclusions: Our findings display an overall excellent DRR of ANA on the long run for both sJIA and AOSD, that may be further optimized by closely monitoring patient’s safety issues and employing this IL-1 inhibitor as a first-line biologic as early as possible. Moreover, ANA allowed a significant drug-sparing effect and showed an overall good safety profile.

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Section: Discussionsupporting

confidence: 94%

Anakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

et al. 2019

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“…During the study period, 20 patients died while hospitalized (mean age 53.9; min-max age 31-80), which a proves relatively low hospital mortality of 2%. A large study based on a nationwide inpatient sample database and covering data on adult hospitalized patients with AOSD in the USA in 2009-2013, reported a mortality rate of 2.6%, and the age of patients who died -62.4 years ± 3.1 [36]. An advanced age was indicated to be a significant predictive factor for the death of patients with macrophage activation syndrome in China [37].…”

Section: Discussionmentioning

confidence: 99%

“…An advanced age was indicated to be a significant predictive factor for the death of patients with macrophage activation syndrome in China [37]. Asian patients were also reported to have a significantly higher in-hospital mortality rate [18]. Additionally, these data may suggest the influence of territorial or race factors on the course of the disease.…”

Section: Discussionmentioning

confidence: 99%

“…was established in Japan at the level of 0.73 and 1.47 per population of 100,000 males and females, respectively [15]. Some studies have shown that females are more affected and account for approximately 60-70% of the patients with AOSD [15,17,18,19]. A retrospective study on the populations of Brittany and Loire in western France, conducted from 1982-1991, showed that the gender ratio was 1.06 (51.6% women and 48.4% men, reference population 1.05) [14].…”

Section: Introductionmentioning

confidence: 99%

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Adult-onset Still’s disease in Poland – a nationwide population-based study

Bogdan

Nitsch–Osuch

Samel-Kowalik

et al. 2021

Ann Agric Environ Med.

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Introduction. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. Materials and method. A retrospective, population-based study was conducted, using data from hospital discharge records compiled by the National Institute of Public Health in 2009-2018. Results. Based on hospitalization records and census data in a group of the 1,050 patients included in the study, women were predominant (60%) and patients' mean and median ages at hospitalization were 42 (95% CI: 40.8-42.8) and 38 years, respectively. The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.30-0.34), and the point prevalence at the end of 2018 was 2.7/100,000. The most common comorbidities were: cardiovascular diseases (14%), diseases of the musculoskeletal system and connective tissue (14%), endocrine, nutritional and metabolic diseases (9%). Conclusions. AOSD is a rare disease Poland with gender and territorial differences in incidence ratek, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

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“…One study reported the mean age of patients who died in hospitals as 62.4 years ± 3.1 and concluded that the older age and associated comorbidities were a predictive factor for the higher incidence of mortality in patients with AOSD. (19) Early identi cation of AOSD in an older patient therefore is more crucial.…”

Section: Discussionmentioning

confidence: 99%

Clinical Spectrum of Adult-Onset Still’s Disease in Patients Presenting At A Tertiary Care Hospital in Pakistan: A 16-Year Follow-Up Study

Riaz¹,

Arshad²,

Baqir³

et al. 2021

Preprint

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Objectives Adult-onset Still’s Disease (AOSD) is a rare systemic auto-inflammatory disorder with a low incidence rate and limited data. This study aims to lay down the clinical and laboratory spectrum, associated complications, and outcomes of patients who presented to a tertiary care center – in order to identify any regional variation in the presentation and enable physicians to develop a thought process in patients without characteristic features. Materials and Methods We reviewed the records of patients from January 2005 - February 2021 who presented with symptoms consistent with AOSD, using the Yamaguchi criteria. The patients’ clinical symptoms, laboratory values, therapeutic management, complications, and outcomes were analysed. Descriptive statistics were produced to illustrate the pattern of the disease. Results The mean age of patients was 37.7 years, with a male to female ratio of 1:1. Fever and arthralgia was seen in 90%, a salmon-coloured rash in 60%, and leukocytosis in 80%. 30% presented with a sore throat and recent lymphadenopathy, 40% with hepatomegaly and/or splenomegaly on imaging, 100% with abnormal liver function tests. Steroids were used as first-line treatment. Most patients did not present with common, previously reported, complications of AOSD. Conclusions The clinical aspects in this study are, to a large part, comparable to those seen in other parts of the world. Thus, a comprehensive analysis of AOSD would enable physicians to broaden their minds and keep it as a top differential in atypical presentations – enhancing patient care by preventing a delay in diagnosis and initiation of prompt management.

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Racial/Ethnic variations in morbidity and mortality in Adult Onset Still's Disease: An analysis of national dataset

Cited by 43 publications

References 23 publications

Anakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

Anakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

Adult-onset Still’s disease in Poland – a nationwide population-based study

Clinical Spectrum of Adult-Onset Still’s Disease in Patients Presenting At A Tertiary Care Hospital in Pakistan: A 16-Year Follow-Up Study

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