Inpatient burden of juvenile dermatomyositis among children in the United States

Abstract

BackgroundJuvenile dermatomyositis (JDM) is a rare autoimmune disease that causes significant morbidity and quality of life impairment. Little is known about the inpatient burden of JDM in the US. Our goal was to determine the prevalence and risk factors for hospitalization with juvenile dermatomyositis and assess inpatient burden of JDM.MethodsData on 14,401,668 pediatric hospitalizations from the 2002–2012 Nationwide Inpatient Sample (NIS) was analyzed. ICD-9-CM coding was used to identify hospitalizations with a diagnosis of JDM.ResultsThere were 909 and 495 weighted admissions with a primary or secondary diagnosis of JDM, respectively. In multivariable logistic regression models with stepwise selection, female sex (logistic regression; adjusted odds ratio [95% confidence interval]) (2.22 [2.05–2.42]), non-winter season (fall: 1.18[1.06–1.33]; spring (1.13 [1.01–1.27]; summer (1.53 [1.37–1.71]), non-Medicaid administered government insurance coverage (2.59 [2.26–2.97]), and multiple chronic conditions (2–5: 1.41[1.30–1.54]; 6+: 1.24[1.00–1.52]) were all associated with higher rates of hospitalization for JDM. The weighted total length of stay (LOS) and inflation-adjusted cost of care for patients with a primary inpatient diagnosis of JDM was 19,159 days and $49,339,995 with geometric means [95% CI] of 2.50 [2.27–2.76] days and $7350 [$6228–$8674], respectively. Costs of hospitalization in primary JDM and length of stay and cost in secondary JDM were significantly higher compared to those without JDM. Notably, race/ethnicity was associated with increased LOS (log-linear regression; adjusted beta [95% confidence interval]) (Hispanic: 0.28 [0.14–0.41]; other non-white: 0.59 [0.31–0.86]) and cost of care (Hispanic: 0.30 [0.05–0.55]).ConclusionJDM contributes to both increased length of hospitalization and inpatient cost of care. Non-Medicaid government insurance was associated with higher rates of hospitalization for JDM while Hispanic and other non-white racial/ethnic groups demonstrated increased LOS and cost of care.

Mapping the current psychology provision for children and young people with juvenile dermatomyositis

“…When asked about their biggest challenge, all three professional groups talked about JDM being a rare disease and patients living far away from treating sites. The issues imposed by working within health-care boundaries and large geographical areas are not specific to the UK, with many patients with JDM in the USA also having issues when accessing care [ 28 ]. It is anticipated that many of the issues raised in this survey (lack of psychology support, rare disease with limited speciality knowledge, lack of time, and large geographical areas) are applicable to other countries in addition to the UK.…”

Paraneoplastic dermatosis in pediatric and adult patients

“…Предрасположенность к ювенильному дерматомиозиту (ЮДМ) может реали зоваться под влиянием факторов окружающей среды: инсоляции, действия лекарственных и инфекционных агентов (бетагемолитического стрептококка группы А, энтеровирусов и вируса Коксаки B) [29,30]. Последний может служить ключом к пониманию того, почему заболевание, повидимому, носит сезонный характер и чаще встречается в теплое время года [31]. Незави симо от влияния различных факторов при ДМ запу скается иммуноопосредованный воспалительный ка скад, приводящий к системной васкулопатии, поражающей эндотелиальные клетки в большинстве тканей пациента [32].…”

Juvenile Dermatomyositis and the Inflammatory Myopathies

“…6 The third may serve a clue as to why there seems to be a seasonal nature to the disease with a higher incidence occurring in nonwinter months. 7 Whether genetically destined or provoked by an environmental or infectious process, an immune-mediated inflammatory cascade is triggered, leading to a systemic vasculopathy affecting the endothelial cells in the majority of a patient's tissues. 8 Early in the disease course, cytokines and interferons cause a small vessel vasculitis.…”