Related Experiment Video
Updated: Nov 17, 2025
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
Clinical Significance of Interstitial Lung Disease and Its Acute Exacerbation in Microscopic Polyangiitis
Hironao Hozumi1, Masato Kono2, Hirotsugu Hasegawa3
1Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Japan.
Background:
Presence of interstitial lung disease (ILD) is thought to be associated with mortality in microscopic polyangiitis (MPA); however, evidence on MPA-ILD remains lacking. Acute exacerbation (AE) refers to rapidly progressive, fatal respiratory deterioration that may develop in patients with various ILDs. No study has investigated the clinical significance of AE in MPA-ILD.
Research Question:
We aimed to determine the clinical picture and prognostic factors, the incidence of AE, and the risk factors in patients with MPA-ILD.
Study Design And Methods:
Eighty-four consecutive patients with MPA-ILD and 95 patients with MPA-non-ILD were analyzed. We also compared 80 patients with MPA-ILD and 80 patients with idiopathic interstitial pneumonia without myeloperoxidase-antineutrophil cytoplasmic antibody positivity (ILD alone), who were matched for age, sex, and chest high-resolution CT scan pattern.
Results:
The MPA-ILD group had a higher frequency of men and smokers and was associated with higher mortality than the MPA-non-ILD group. The matched MPA-ILD group had a higher mortality rate than the matched ILD alone group. There was no significant difference in AE incidence between the matched MPA-ILD and ILD alone groups (1-year AE cumulative incidence rate, 7.5% and 5.2%, respectively; P = .75). In the MPA-ILD group, a lower percent predicted FVC (%FVC) was independently associated with a higher mortality rate (hazard ratio [HR], 0.96 per 1% increase; P < .01) and a higher AE incidence rate (HR, 0.96 per 1% increase; P = .01). On multivariable Cox regression analysis with time-dependent covariates, developing AE during their clinical course was strongly associated with shorter survival (HR, 17.1; P < .001).
Interpretation:
MPA-ILD represented a distinct phenotype with poor prognosis. Lower %FVC was an independent prognostic factor. Patients with lower %FVC had a risk of developing AE, which was a strong prognostic determinant. The specific management for MPA-ILD and AE should be established.
Insights
Microscopic polyangiitis with interstitial lung disease (MPA-ILD) indicates a poor prognosis. Lower lung function (FVC) predicts mortality and acute exacerbations (AE), which significantly shorten survival in MPA-ILD patients.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Immunology
Background:
- Interstitial lung disease (ILD) presence is linked to mortality in microscopic polyangiitis (MPA), but evidence is limited.
- Acute exacerbations (AE) are severe respiratory events in ILD, yet their significance in MPA-ILD is unstudied.
Purpose of the Study:
- To define the clinical characteristics and prognosis of MPA-ILD.
- To investigate the incidence and risk factors for AE in MPA-ILD.
- To identify prognostic factors for mortality and AE in MPA-ILD.
Main Methods:
- Retrospective analysis of 84 MPA-ILD patients and 95 MPA-non-ILD patients.
- Comparison of 80 MPA-ILD patients with 80 age/sex/CT-matched idiopathic interstitial pneumonia (ILD alone) patients.
- Multivariable Cox regression analysis to identify prognostic factors.
Main Results:
- MPA-ILD patients had higher mortality than MPA-non-ILD and ILD alone groups.
- AE incidence did not differ significantly between matched MPA-ILD and ILD alone groups.
- Lower percent predicted FVC (%FVC) independently predicted higher mortality and AE risk in MPA-ILD. Developing AE strongly predicted shorter survival (HR 17.1).
Conclusions:
- MPA-ILD is a distinct phenotype with a poor prognosis.
- Reduced %FVC is a key prognostic factor for mortality and AE development.
- AE is a critical determinant of survival in MPA-ILD, necessitating specific management strategies.
Related Concept Videos
01:31Pulmonary Tuberculosis III
The first classification is based on the development of the disease, and it includes the following categories:
01:28Pulmonary Tuberculosis II
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
01:27Acute Pyelonephritis I: Introduction
01:29Pulmonary Embolism I: Introduction
01:29Pulmonary Tuberculosis I
Causative Organism
The primary infectious agent causing tuberculosis is Mycobacterium tuberculosis, a slow-growing, acid-fast, aerobic rod that exhibits sensitivity to heat and ultraviolet light. Instances of Mycobacterium bovis and Mycobacterium avium contributing to the development of TB infection are rare.
Mode of...
01:30Peripheral Artery Disease I: Introduction