ArticleBMC pulmonary medicine2022
Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases.
Article in BMC pulmonary medicine, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers, 1 of them a synthesis that pooled it.
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10 citing papers in PubMed, 1 synthesis or guideline pooled it, 19 citations in OpenAlex.
- Incidence, risk factors, and prognosis of acute exacerbation of rheumatoid arthritis-associated interstitial lung disease: a systematic review and meta-analysis.BMC pulmonary medicine · 2023Pooled it
- Risk stratification of acute exacerbations by autoantibodies in patients with idiopathic interstitial pneumonia.Scientific reports · 2025Article
- Observational
- Potential of phosphodiesterase 4B inhibitors in the treatment of interstitial lung disease associated with autoimmune diseases.Clinical and experimental rheumatology · 2025Review
- Acute exacerbations in patients with progressive pulmonary fibrosis.ERJ open research · 2024Article
- High resolution computed tomography in systemic sclerosis: From diagnosis to follow-up.Rheumatology and immunology research · 2024Article
- Usual interstitial pneumonia is the predominant histopathology in patients with systemic sclerosis receiving a lung transplant.Clinical and experimental rheumatology · 2023Article
- Re-hospitalisation predicts poor prognosis after acute exacerbation of interstitial lung disease.BMC pulmonary medicine · 2023Article
- Pathological Roles of Pulmonary Cells in Acute Lung Injury: Lessons from Clinical Practice.International journal of molecular sciences · 2022Review
- Association between acute exacerbation and progressive pulmonary fibrosis in interstitial lung disease: a retrospective cohort study.Therapeutic advances in respiratory diseaseArticle
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18 authors at 3 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundAcute exacerbation (AE) of systemic autoimmune disease-related interstitial lung diseases (SAID-ILD) is less common than AE of idiopathic pulmonary fibrosis (IPF) and the details of AE-SAID-ILD have not been elucidated, but the prognosis is similarly devastating. This study was undertaken to determine the incidences of AE-ILD in each SAID and to elucidate the proportion of progressive fibrosing (PF)-ILD in AE-SAID-ILD.
methodsWe retrospectively analysed data for patients with SAID-ILD who were diagnosed and observed at our hospital between 1999 and 2020.
resultsTwo hundred and thirty-two patients with SAID-ILD were enrolled, with a mean observation period of 100.2 months. AE-SAID-ILD was found in 25 patients (10.78%), mainly in patients with RA (17 patients, 68%) and elderly male patients with a smoking history. The overall incidence of AE-SAID-ILD was 1.29%/person-year, and the incidence for each SAID was as follows: RA 2.193, microscopic polyarteritis (MPA) 3.203, systemic sclerosis (SSc) 2.277, primary Sjögren syndrome 0.426, and polymyositis/dermatomyositis 0.222. The incidence of AE of RA/MPA/SSc-ILD was significantly higher than that of other AE-SAID-ILD (p < 0.001). Five of 25 patients (20%) fulfilled the criteria for PF-ILD. The 90-day survival rate was 48.0%, and a higher neutrophil count at AE (HR 13.27, 95%CI 2.447-246, p = 0.001) and early commencement of long-duration direct haemoperfusion with a polymyxin B-immobilised fibre column (HR 0.105, 95%CI 0.005-0.858, p = 0.035) were significant prognostic factors.
conclusionsThe incidence of AE-SAID-ILD was significantly higher in patients with RA, MPA, or SSc than in patients with other SAID. Furthermore, even in patients with AE-SAID-ILD, the proportion of PF-ILD just before AE was not high (20%).
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