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Prediction model for respiratory-related mortality in microscopic polyangiitis with interstitial lung disease:
Shogo Matsuda1, Takuya Kotani1, Ayana Okazaki1
1Department of Internal Medicine IV, Division of Rheumatology, Osaka Medical and Pharmaceutical University, Osaka, Japan.
Objective:
This study aimed to establish prediction models for respiratory-related mortality in microscopic polyangiitis (MPA) complicated by interstitial lung disease (ILD) using clinical characteristics.
Methods:
We enrolled patients with MPA with ILD between May 2005 and June 2021 in a multicentre cohort of Japanese patients with MPA (REVEAL cohort). We evaluated the demographic, clinical, laboratory, radiological findings, treatments and the presence of honeycombing 1 cm above the diaphragm using chest high-resolution CT (HRCT) on admission. We explored the risk factors predictive of respiratory-related mortality.
Results:
Of 115 patients, 26 cases died of respiratory-related diseases during a median follow-up of 3.8 years. Eighteen patients (69%) died due to respiratory infection, three (12%) had diffuse alveolar haemorrhage, and five (19%) had exacerbation of ILD. In univariate analysis, older age, lower percent forced vital capacity (%FVC), lower percent diffusing capacity of carbon monoxide (%DLCO), and the presence of honeycombing in the right lower lobe were identified as risk factors. Additionally, in multivariate analysis adjusted for age and treatment, %FVC, %DLCO and the presence of honeycombing in the right lower lobe were independently associated with respiratory-related mortality. We created prediction models based on the values of %FVC, %DLCO and presence of honeycombing on chest HRCT (termed "MPF model"). The 5-year respiratory-related death-free rate was significantly different between patients with MPA with ILD stratified by the number of risk factors based on the MPF model.
Conclusions:
Our study indicates that the MPF model may help predict respiratory-related death in patients with MPA with ILD.
Insights
A new MPF model can predict respiratory death in patients with microscopic polyangiitis (MPA) and interstitial lung disease (ILD). Key factors include lung function (FVC, DLCO) and honeycombing on CT scans.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Radiology
Background:
- Microscopic polyangiitis (MPA) with interstitial lung disease (ILD) carries a significant risk of respiratory mortality.
- Identifying predictive factors for mortality is crucial for managing patients with MPA-ILD.
Purpose of the Study:
- To develop and validate a prediction model for respiratory-related mortality in patients with MPA-ILD.
- To identify clinical and radiological predictors of respiratory death in this patient population.
Main Methods:
- A multicentre cohort of 115 Japanese patients with MPA-ILD was analyzed.
- Clinical, laboratory, and chest HRCT findings, including honeycombing, were assessed.
- Risk factors for respiratory-related mortality were identified using univariate and multivariate analyses.
Main Results:
- Respiratory-related mortality occurred in 26 patients over a median follow-up of 3.8 years.
- Risk factors identified included older age, lower %FVC, lower %DLCO, and right lower lobe honeycombing.
- The developed MPF model, incorporating %FVC, %DLCO, and honeycombing, showed significant stratification of 5-year death-free rates.
Conclusions:
- The MPF model demonstrates potential utility in predicting respiratory-related death in MPA-ILD patients.
- Lung function parameters (%FVC, %DLCO) and specific HRCT findings (honeycombing) are key predictors.
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