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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Long-term clinical course and outcomes of patients with microscopic polyangiitis-associated interstitial lung disease
Min Jee Kim1, Donghee Lee2, Jooae Choe3
1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Abstract:
Background: Interstitial lung disease (ILD) is a significant complication associated with microscopic polyangiitis (MPA) that has a poor prognosis. However, the long-term clinical course, outcomes, and prognostic factors of MPA-ILD are not well defined. Hence, this study aimed to investigate the long-term clinical course, outcomes, and prognostic factors in patients with MPA-ILD. Methods: Clinical data of 39 patients with MPA-ILD (biopsy proven cases, n = 6) were retrospectively analyzed. High resolution computed tomography (HRCT) patterns were assessed based on the 2018 idiopathic pulmonary fibrosis diagnostic criteria. Acute exacerbation (AE) was defined as the worsening of dyspnea within 30 days, with new bilateral lung infiltration that is not fully explained by heart failure or fluid overload and that does not have identified extra-parenchymal causes (pneumothorax, pleural effusion, or pulmonary embolism). Results: The median follow-up period was 72.0 months (interquartile range: 44-117 months). The mean age of the patients was 62.7 years and 59.0% were male. Usual interstitial pneumonia (UIP) and probable usual interstitial pneumonia patterns on high resolution computed tomography were identified in 61.5 and 17.9% of the patients, respectively. During the follow-up, 51.3% of patients died, and the 5- and 10-year overall survival rates were 73.5% and 42.0%, respectively. Acute exacerbation occurred in 17.9% of the patients. The non-survivors had higher neutrophil counts in bronchoalveolar lavage (BAL) fluid and more frequent acute exacerbation than the survivors. In the multivariable Cox analysis, older age (hazard ratio [HR], 1.07; 95% confidence interval [CI], 1.01-1.14; p = 0.028) and higher BAL counts (HR, 1.09; 95% CI, 1.01-1.17; p = 0.015) were found to be the independent prognostic factors associated with mortality in patients with MPA-ILD. Conclusion: During the 6 years-follow-up, about half of patients with MPA-ILD died and approximately one-fifth experienced acute exacerbation. Our results suggest that older age and higher BAL neutrophil counts mean poor prognosis in patients with MPA-ILD.
Insights
Microscopic polyangiitis with interstitial lung disease (MPA-ILD) has a poor prognosis, with nearly half of patients dying within six years. Older age and elevated bronchoalveolar lavage neutrophil counts indicate a worse outcome for MPA-ILD patients.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Internal Medicine
Background:
- Interstitial lung disease (ILD) is a serious complication of microscopic polyangiitis (MPA).
- The long-term prognosis and prognostic factors for MPA-ILD remain poorly understood.
- This study addresses the need for clarity on the clinical course and outcomes of MPA-ILD.
Purpose of the Study:
- To investigate the long-term clinical course and outcomes of patients diagnosed with MPA-ILD.
- To identify independent prognostic factors associated with mortality in MPA-ILD.
- To provide data that can inform clinical management and patient counseling.
Main Methods:
- Retrospective analysis of clinical data from 39 MPA-ILD patients.
- High-resolution computed tomography (HRCT) patterns assessed using 2018 idiopathic pulmonary fibrosis criteria.
- Definition of acute exacerbation (AE) based on rapid worsening of respiratory symptoms and new lung infiltrates.
Main Results:
- Median follow-up was 72 months; 51.3% of patients died.
- 5- and 10-year overall survival rates were 73.5% and 42.0%, respectively.
- Older age and higher bronchoalveolar lavage (BAL) neutrophil counts were independent predictors of mortality.
Conclusions:
- MPA-ILD carries a significant mortality risk, with approximately half of patients dying within six years.
- Acute exacerbations occurred in 17.9% of patients during the follow-up period.
- Older age and elevated BAL neutrophil counts are associated with poor prognosis in MPA-ILD.
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