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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Clinical features and outcomes of children's interstitial lung disease accompanied with connective tissue disease: A
Gaoli Jiang1, Jingyi Xia1, Quanli Shen2
1Department of Respiratory Medicine, Children's Hospital of Fudan University, Shanghai, 201102, China.
Insights
Childhood interstitial lung disease (chILD) associated with connective tissue disease (CTD) presents significant challenges. Early CTD assessment in chILD is crucial, as complications like ARDS predict poor outcomes, while specific treatments offer protection.
Area of Science:
- Pediatric Pulmonology
- Rheumatology
- Medical Complexity
Background:
- Childhood interstitial lung disease (chILD) associated with connective tissue disease (CTD) is medically complex.
- Pediatricians face challenges in managing these intertwined conditions.
Purpose of the Study:
- To analyze clinical characteristics, laboratory findings, PFTs, treatments, and outcomes in patients with CTD-chILD.
- To identify predictors of mortality and protective factors in CTD-chILD.
Main Methods:
- Prospective study analyzing clinical data, lab results, PFTs, treatments, and outcomes.
- Utilized Cox univariate and multivariate analyses to identify survival predictors.
Main Results:
- Median age at diagnosis was 7 years; 29.4% had rapidly progressive chILD (RP-chILD) with high mortality.
- Decreased diffusion capacity was common (>70%); diagnosis interval was longer for chILD preceding CTD, associated with higher mortality.
- Acute respiratory distress syndrome (ARDS) predicted death in CTD-chILD, while IVIG, corticosteroids, and immunosuppressants were protective.
Conclusions:
- Assess for CTD in chILD patients due to increased mortality risk with longer diagnosis intervals.
- ARDS is a poor prognostic indicator in CTD-chILD; combination therapy offers protection.
Background:
Medical complexity of childhood interstitial lung disease (chILD) with connective tissue disease (CTD) poses a considerable challenge to pediatricians.
Methods:
Clinical characteristics, laboratory findings, pulmonary function tests (PFTs), treatments and outcomes obtained for patients with CTD-chILD were analyzed in a prospective study.
Results:
Patients' median age at diagnosis was 7 years old. About 29.4% (15/51) suffered rapidly progressive childhood ILD (RP-chILD) with a high mortality rate (33.3%, 5/15), and the incidence of RP-chILD in juvenile idiopathic inflammatory myopathies was as high as 41.6% and the mortality rate was 30% (3/10). More than 70% patients had decreased diffusion capacity. The mean interval from symptoms-onset to diagnosis was 11.3 months. Compared to chILD with known CTD, the chILD proceeded CTD had a longer diagnosis interval, higher mortality, hospital stays and costs (P < 0.05). Lung imaging (33.3%) and lung function (72.7%) were partially reversible. The average survival time was 68.6 months. Cox univariate analysis showed that HRCT score ≥3, experiencing RP-chILD, cyanosis, acute respiratory distress syndrome (ARDS) and CD4 T cell <200 were significant predictors of death for chILD, whereas Cox multivariate analysis showed that ARDS was significant predictor of death for CTD-chILD, while IVIG support combined with corticosteroids and immunosuppressants was a protective factor.
Conclusions:
Care providers should conduct an assessment for CTD in chILD as a longer interval between the diagnosis of chILD and the CTD is associated with increased mortality. Complications as ARDS predict poor outcome in CTD-chILD, while IVIG support combined with corticosteroids and immunosuppressants is a protective factor.
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