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Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis
- Source :
- Rheumatology (Oxford, England). 54(5)
- Publication Year :
- 2013
-
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 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.
- Subjects :
- Male
Pathology
medicine.medical_specialty
Interferon-Induced Helicase, IFIH1
Adolescent
medicine.disease_cause
behavioral disciplines and activities
Dermatomyositis
Autoimmunity
DEAD-box RNA Helicases
Rheumatology
Japan
Predictive Value of Tests
medicine
Humans
Pharmacology (medical)
Child
Juvenile dermatomyositis
Retrospective Studies
biology
business.industry
Intensive treatment
Mucin-1
Interstitial lung disease
Interleukin-18
Infant
respiratory system
medicine.disease
Prognosis
respiratory tract diseases
Antibodies, Anti-Idiotypic
body regions
Ferritin
Child, Preschool
Ferritins
biology.protein
Disease Progression
Creatine kinase
Female
Antibody
business
Complication
Lung Diseases, Interstitial
Biomarkers
Subjects
Details
- ISSN :
- 14620332
- Volume :
- 54
- Issue :
- 5
- Database :
- OpenAIRE
- Journal :
- Rheumatology (Oxford, England)
- Accession number :
- edsair.doi.dedup.....526e3123426bb9ca3b8e657664ac4ba3