Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis.
Rheumatology (Oxford)
; 54(5): 784-91, 2015 May.
Article
em En
| MEDLINE
| ID: mdl-25288783
ABSTRACT
OBJECTIVE:
Rapidly progressive interstitial lung disease (RP-ILD) is a rare but potentially fatal complication of JDM. The aim of this study was to establish markers for the prediction and early diagnosis of RP-ILD associated with JDM.METHODS:
The clinical records of 54 patients with JDM were retrospectively reviewed 10 had RP-ILD (7 died, 3 survived), 19 had chronic ILD and 24 were without ILD. Routine tests included a high-resolution CT (HRCT) scan of the chest and measurement of serum levels of creatine phosphokinase, ferritin and Krebs von den Lungen-6 (KL-6). Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies and IL-18 levels were measured by ELISA.RESULTS:
No differences were found in the ratio of juvenile clinically amyopathic DM between the three groups. Initial chest HRCT scan findings were variable and could not distinguish between RP-ILD and chronic ILD. Anti-MDA5 antibodies were positive in all 8 patients with RP-ILD and 10 of 14 with chronic ILD, but none of the patients without ILD. Serum levels of anti-MDA5 antibody, ferritin, KL-6 and IL-18 were significantly higher in the RP-ILD group than in the chronic ILD and non-ILD groups. Serum levels of IL-18 positively correlated with serum KL-6 (R = 0.66, P < 0.001).CONCLUSION:
High serum levels of IL-18, KL-6, ferritin and anti-MDA5 antibodies (e.g. >200 units by ELISA) are associated with RP-ILD. These can be used as an indication for early intensive treatment. Both alveolar macrophages and autoimmunity to MDA5 are possibly involved in the development of RP-ILD associated with JDM.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Doenças Pulmonares Intersticiais
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Progressão da Doença
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Dermatomiosite
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
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Screening_studies
Limite:
Adolescent
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
País como assunto:
Asia
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article