{Reference Type}: Journal Article
{Title}: Prediction model for respiratory-related mortality in microscopic polyangiitis with interstitial lung disease: multicentre REVEAL cohort study.
{Author}: Matsuda S;Kotani T;Okazaki A;Nishioka D;Watanabe R;Gon T;Manabe A;Shoji M;Kadoba K;Hiwa R;Yamamoto W;Hashimoto M;Takeuchi T;
{Journal}: Rheumatology (Oxford)
{Volume}: 63
{Issue}: 6
{Year}: 2024 May 3
{Factor}: 7.046
{DOI}: 10.1093/rheumatology/kead444
{Abstract}: <B>OBJECTIVE: </B>This study aimed to establish prediction models for respiratory-related mortality in microscopic polyangiitis (MPA) complicated by interstitial lung disease (ILD) using clinical characteristics.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>Our study indicates that the MPF model may help predict respiratory-related death in patients with MPA with ILD.