Abstract:Certain classes of environmental agents that have been associated with the initiation of DM, including sun exposure and medications, may also play a role in disease flares.
“…Photosensitivity is reported in nearly half of patients with juvenile myositis (JM) , and exacerbations of skin disease following sun exposure have been described. Once DM is established, UV radiation appears to be a strong trigger: laboratory testing of non‐irradiated skin of adults with DM determined increased sensitivity to ultraviolet B (UVB) radiation compared to healthy controls, many of whom also reported photosensitivity and disease exacerbation following sun exposure , and questionnaire data from patients with DM and JM suggested that UV exposure is an important environmental exposure correlated with disease flares .This study explores the association between the environmental factor ultraviolet (UV) radiation and disease severity outcomes in a large registry of patients with juvenile dermatomyositis (DM) by integrating clinical and demographic data from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry with historical National Aeronautics and Space Administration satellite measurements.Mean UV index (UVI) exposure in the month prior to disease onset was associated with development of calcinosis; however, the directionality of this relationship was dependent on race.Mean UVI exposure was not associated with other features representative of severe disease, including skin ulceration, a Childhood Health Assessment Questionnaire score >1, use of biologics or nonmethotrexate disease‐modifying antirheumatic drugs, use of intravenous immunoglobulin, or persistent skin disease, muscle weakness, or steroid use beyond 2 years of disease duration.These results further our knowledge of the role of UV radiation in the clinical course of juvenile DM and highlight the need for clinicians and researchers to be aware of the complex interplay of genes and environment in the clinical phenotypes of children with juvenile DM.…”
Objective. Ultraviolet (UV) radiation is considered to be an important environmental factor in the clinical course of children with juvenile dermatomyositis (DM). We aimed to evaluate the association between UV radiation and severe disease outcomes in juvenile DM.Methods. This is a cross-sectional study of patients with juvenile DM enrolled in the US multicenter Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry from 2010 to 2015. The mean UV index (UVI) in the calendar month prior to symptom onset in each subject's zip code was calculated from daily satellite solar noon measurements. Multivariable logistic regression was used to model the relationship between the mean UVI and calcinosis as well as other outcomes of severe disease. Covariates included sex, race, age, time to diagnosis, disease duration, and latitude.Results. In a multivariable model, there was no association between the mean UVI and calcinosis. African American race was associated with a 3-fold greater odds of calcinosis. However, there was a significant statistical interaction between race and mean UVI. Accounting for this interaction, the odds of calcinosis markedly decreased in African American subjects and steadi ly increased in non-African American subjects over a range of increasing the mean UVI. Higher mean UVI was associated with decreased odds of using biologics or nonmethotrexate disease-modifying antirheumatic drugs and skin ulceration.Conclusion. We described a novel association between UV radiation, calcinosis, and race in a large cohort of patients with juvenile DM. This study furthers our knowledge of the role of UV radiation in the clinical course of juvenile DM and highlights the complex interplay between genes and environment in the clinical phenotypes and development of calcinosis in children with juvenile DM.
“…Photosensitivity is reported in nearly half of patients with juvenile myositis (JM) , and exacerbations of skin disease following sun exposure have been described. Once DM is established, UV radiation appears to be a strong trigger: laboratory testing of non‐irradiated skin of adults with DM determined increased sensitivity to ultraviolet B (UVB) radiation compared to healthy controls, many of whom also reported photosensitivity and disease exacerbation following sun exposure , and questionnaire data from patients with DM and JM suggested that UV exposure is an important environmental exposure correlated with disease flares .This study explores the association between the environmental factor ultraviolet (UV) radiation and disease severity outcomes in a large registry of patients with juvenile dermatomyositis (DM) by integrating clinical and demographic data from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry with historical National Aeronautics and Space Administration satellite measurements.Mean UV index (UVI) exposure in the month prior to disease onset was associated with development of calcinosis; however, the directionality of this relationship was dependent on race.Mean UVI exposure was not associated with other features representative of severe disease, including skin ulceration, a Childhood Health Assessment Questionnaire score >1, use of biologics or nonmethotrexate disease‐modifying antirheumatic drugs, use of intravenous immunoglobulin, or persistent skin disease, muscle weakness, or steroid use beyond 2 years of disease duration.These results further our knowledge of the role of UV radiation in the clinical course of juvenile DM and highlight the need for clinicians and researchers to be aware of the complex interplay of genes and environment in the clinical phenotypes of children with juvenile DM.…”
Objective. Ultraviolet (UV) radiation is considered to be an important environmental factor in the clinical course of children with juvenile dermatomyositis (DM). We aimed to evaluate the association between UV radiation and severe disease outcomes in juvenile DM.Methods. This is a cross-sectional study of patients with juvenile DM enrolled in the US multicenter Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry from 2010 to 2015. The mean UV index (UVI) in the calendar month prior to symptom onset in each subject's zip code was calculated from daily satellite solar noon measurements. Multivariable logistic regression was used to model the relationship between the mean UVI and calcinosis as well as other outcomes of severe disease. Covariates included sex, race, age, time to diagnosis, disease duration, and latitude.Results. In a multivariable model, there was no association between the mean UVI and calcinosis. African American race was associated with a 3-fold greater odds of calcinosis. However, there was a significant statistical interaction between race and mean UVI. Accounting for this interaction, the odds of calcinosis markedly decreased in African American subjects and steadi ly increased in non-African American subjects over a range of increasing the mean UVI. Higher mean UVI was associated with decreased odds of using biologics or nonmethotrexate disease-modifying antirheumatic drugs and skin ulceration.Conclusion. We described a novel association between UV radiation, calcinosis, and race in a large cohort of patients with juvenile DM. This study furthers our knowledge of the role of UV radiation in the clinical course of juvenile DM and highlights the complex interplay between genes and environment in the clinical phenotypes and development of calcinosis in children with juvenile DM.
“…3 The predisposition for JDM may be flared by environmental factors such as sun exposure, medications, or certain infections. 4 Suspected organisms inciting JDM are group A beta hemolytic streptococci, 5 enterovirus, 2 and Coxsackie B virus. 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.…”
The inflammatory myopathies comprise disorders of immune-mediated muscle injury. The histopathology and clinical features help distinguish them. Juvenile dermatomyositis (JDM) is the most common form of myositis in children and adolescents. Children with JDM present with proximal muscle weakness and characteristic rashes. The presentation is similar in children and adults, but JDM is a primary disorder and the adult form often is concerning for a paraneoplastic syndrome. Proximal muscle weakness occurs with dermatomyositis, polymyositis, and immune-mediated necrotizing myopathy, but the latter two conditions have no dermatologic findings or distinct tissue changes which set them apart from dermatomyositis. Inclusion body myositis, also included in the inflammatory myopathies, presents with more distal involvement, and microscopically exhibits identifiable rimmed vacuoles. We review key features of these disorders, focusing in more detail on JDM because it is more often encountered by the child neurologist.
“…In this sense, several immunogenetic risk factors, including certain class-2 human leukocyte antigen (HLA) alleles, have been implicated in dermatomyositis pathogenesis [23]. Studies suggest that exposure to ultraviolet light may also be considered an important risk factor for the development of dermatomyositis [24].…”
Section: Dermatomyositismentioning
confidence: 99%
“…Histopathological hallmarks of polymyositis include invasion of endomysial cytotoxic CD8 T cells and widespread upregulation of class I MHC in muscle fibers [2,24]. Polymyositis is a chronic, degenerative disease that has no cure.…”
Inflammatory myopathies, also called idiopathic inflammatory myopathy or myositis, are rare conditions characterized by the involvement of various organs in addition to muscle tissue. These changes can lead to severe impairments and adversely impact the quality of life of affected individuals. The diagnosis and treatment of inflammatory myopathies involve the participation of an interdisciplinary team, due to the complexity of the disease and the high variety of possible signs and symptoms. In this chapter we will discuss the epidemiology and characteristics of the main subtypes of inflammatory myopathies, such as polymyositis, dermatomyositis, necrotizing myopathy, overlap myositis, and myositis of inclusion bodies. Next, we will discuss the existence of crosstalk between inflammatory processes in the oral cavity and their consequences on skeletal muscle. As oral inflammation can increase infiltration of macrophages in muscle tissue and this increase is related to the production of proinflammatory cytokines in this tissue, these cytokines can cause muscle weakness. It is important to consider the prevention of chronic inflammatory processes in order to maintain muscle integrity or even prevent the worsening of the clinical condition of patients with inflammatory muscle diseases.
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