Dataset Information

Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases.

ABSTRACT:

Background

Acute 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.

Methods

We retrospectively analysed data for patients with SAID-ILD who were diagnosed and observed at our hospital between 1999 and 2020.

Results

Two 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.

Conclusions

The 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%).

SUBMITTER: Enomoto N

PROVIDER: S-EPMC9608932 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature

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Publications

Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases.

Enomoto Noriyuki N Naoi Hyogo H Mochizuka Yasutaka Y Isayama Takuya T Tanaka Yuko Y Fukada Atsuki A Aono Yuya Y Katsumata Mineo M Yasui Hideki H Mori Kazutaka K Karayama Masato M Hozumi Hironao H Suzuki Yuzo Y Furuhashi Kazuki K Fujisawa Tomoyuki T Inui Naoki N Nakamura Yutaro Y Suda Takafumi T

BMC pulmonary medicine 20221026 1

<h4>Background</h4>Acute 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.<h4>Methods</h4>We retrospectively analysed data for patien ...[more]

PMID: 36289542

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Project description:BackgroundAdvanced pulmonary sarcoidosis causes significant morbidity and can lead to death. Large trials demonstrated efficacy of antifibrotics in patients with progressive fibrosing interstitial lung diseases (PF-ILD), including a few with sarcoidosis. To date, little is known about this progressive fibrosing phenotype in sarcoidosis. Diffusion capacity of carbon monoxide (DLCO) may be a useful functional marker to screen for advanced pulmonary sarcoidosis. In this study, we describe a cohort with advanced pulmonary sarcoidosis and we gain insights in the progressive fibrosing phenotype in sarcoidosis.MethodsPatients with sarcoidosis and a DLCO < 50% predicted were included in this retrospective cohort study. First measurement of DLCO < 50% predicted was the baseline. Lung function data, HRCT, pulmonary hypertension (PH) and mortality were collected. Patients with > 10% fibrosis on HRCT meeting the criteria for ILD-progression within 24 months were labelled as PF-ILD. With Cox-regression analysis predictors of mortality were established.Results106 patients with a DLCO < 50% predicted were included. Evolution of forced vital capacity (FVC) varied widely between patients from - 34% to + 45% after 2 years follow-up, whereas change in DLCO varied between - 11% and + 26%. Fourteen patients (15%) met the PF-ILD criteria, of whom 6 (43%) died within 10 years versus 10 (13%) in the non PF-ILD group (p = 0.006). PH was present 12 (11%), 56 (53%) demonstrated > 10% fibrosis on HRCT. Independent predictors of mortality and lung transplantation in the whole cohort are PH, PF-ILD and UIP-like pattern.ConclusionIn conclusion, within this group with advanced pulmonary sarcoidosis disease course varied widely from great functional improvement to death. PF-ILD patients had higher mortality rate than the mortality in the overall pulmonary sarcoidosis group. Future research should focus on the addition of antifibrotics in these patients. Trial registration retrospectively registered.

Project description:IntroductionAcute exacerbation (AE) is a devastating phenomenon and reported to be complicated with systemic autoimmune disease-associated interstitial lung disease (ILD). The aim of this study was to investigate the incidence and prognosis of AE of systemic autoimmune disease-ILD and clarify relevant clinical information predictive of these outcomes.MethodThis study was designed as a systematic review and meta-analysis. A primary study except for a case report, which reported the incidence and/or prognosis of AE of systemic autoimmune disease-ILD, was eligible for the review. Electronic databases such as Medline and EMBASE were searched from 2002 through 23 February 2020. Two reviewers independently selected eligible reports and extracted relevant data. Risk of bias of individual studies was assessed similarly. The incidence and prognosis of the disease were analysed qualitatively. Univariate results of risk and prognostic factors were combined if feasible.ResultsOut of a total of 2662 records, 24 studies were eligible. A total of 420 subjects with 45.7% of men developed AE of systemic autoimmune disease-ILD and the two major underlying systemic autoimmune diseases were rheumatoid arthritis (34.2%) and polymyositis/dermatomyositis (31.9%). The frequency ranged from 4.3 to 32.9% with the incident rate being 3.19 and 5.77 per 100 patient-years and all-cause mortality was between 30.0 and 58.3% at 90 days. Age at initial presentation was significantly associated with the development of AE of systemic autoimmune disease-ILD with an HR of 1.22 (95%CI 1.05-1.50) while a percentage of predicted diffusing capacity of the lung for carbon monoxide (%DLCO) was also significantly associated with the development of the disease with an HR of 0.95 (95%CI 0.90-1.00) and an OR of 0.97 (95%CI 0.95-0.99). Partial pressure of arterial oxygen/fraction of inspired oxygen ratio (PaO2/FiO2) at AE was significantly associated with all-cause mortality of AE of systemic autoimmune disease-ILD with an HR of 0.99 (95%CI 0.98-0.99).ConclusionAE of systemic autoimmune disease-ILD was not uncommon and demonstrated dismal prognosis. Age at initial presentation and %DLCO were deemed as risk factors while PaO2/FiO2 at AE was considered as a prognostic factor of the disease. Registration CRD42019138941.

Dataset Information

Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases.

Background

Methods

Results

Conclusions

Publications

Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases.

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