Fatores de risco associados à calcinose na dermatomiosite juvenil

Abstract

Objective: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis. Methods:A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. Conclusions:Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.

Juvenile dermatomyositis in South African children is characterised by frequent dystropic calcification: a cross sectional study

“…In Scandinavia, only 20% developed calcinosis in association with long term disease duration [16]. Our series is in contrast to these reports as well as a study from Brazil in which calcinosis was noted only in the more severe cases, and cardiac involvement was identified being independently associated with calcinosis [17]. However, we had only one child with cardiac disease in this series.…”

The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis

“…Patients with a chronic or polycyclic course, and therefore with longer duration of active disease, may be more likely to develop calcinosis despite adequate therapy [7,8,24]. The presence of cardiac involvement and the use of one or more immunosuppressive therapies (other than oral corticosteroids) have been associated with the development of calcinosis [25]. …”

Developments in the Classification and Treatment of the Juvenile Idiopathic Inflammatory Myopathies

“…Patients with a chronic illness course and poor prognosis might also have cutaneous or gastrointestinal ulcerations, or less commonly pulmonary or gastrointestinal tract air leaks (pneumomediastinum or pneumatosis intestinalis), indicating severe vasculopathy 14 . Calcinosis occurs in 20–40% of patients 5;9;15 , with a lower prevalence in patients treated more aggressively who enter remission more rapidly 16 . Calcium and phosphate precipitate in the subcutaneous tissues and muscles as carbonate apatite, perhaps as a result of a loss of mineralization inhibitors 17 .…”

“…Calcium and phosphate precipitate in the subcutaneous tissues and muscles as carbonate apatite, perhaps as a result of a loss of mineralization inhibitors 17 . Risk factors for calcinosis include delay to diagnosis, cardiac involvement, the need for additional immunosuppressive therapy, as well as a prolonged and/or severe illness course 15 . Another complication of JDM, lipodystrophy, occurs in up to 10% of JDM patients and is characterized by progressive loss of subcutaneous fat in a localized, partial (i.e., only in the extremities), or widespread distribution.…”