Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study

Cao, Mengshu; Sheng, Jinhua; Qiu, Xiaohua; Wang, Dandan; Wang, Dongmei; Wang, Yang; Xiao, Yonglong; Cai, Hourong

doi:10.1186/s12890-019-0960-1

Cited by 43 publications

(37 citation statements)

References 42 publications

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“…demonstrated that the cut-off level of ΔLDH level of > 80 IU/L, as measured within 2 weeks, was related to poor prognosis. Furthermore, changes in LDH were reportedly associated with AE-related poor prognosis in patients with connective tissue disease associated with interstitial pneumonia [23]. Our study suggests that LDH change was useful as a prognostic factor.…”

Section: Discussionsupporting

confidence: 50%

Serum Lactate Dehydrogenase Level As a Predictor Of Survival For Patients With Acute Exacerbation of Idiopathic Interstitial Pneumonias Undergoing Polymyxin B-Immobilized Fiber Column-Direct Hemoperfusion

Kato

Nakazawa

Akimoto

et al. 2021

Preprint

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BackgroundAcute exacerbation of chronic fibrosing idiopathic interstitial pneumonias (AE-IIPs) is known to be associated with a poor prognosis. In Japan, polymyxin B-immobilized fiber column-direct hemoperfusion (PMX-DHP) therapy is often used along with corticosteroids for the treatment of patients with AE-IIPs. However, the serum marker that predicts treatment response to PMX-DHP is still unknown. Our aim was to evaluate the serum marker that predicts the outcome in patients with AE-IIPs undergoing PMX-DHP therapy.MethodsWe retrospectively collected the medical records of 104 patients who developed AE-IIPs for the first time and visited Juntendo University Hospital between April 2009 and July 2020. Among these patients, 33 patients who received PMX-DHP were identified. Among the patients who received PMX-DHP, 18 patients who survived for over 30 days since the initiation of PMX-DHP therapy were classified into the “survival group,” and 15 patients who succumbed to the condition in less than 30 days were categorized into the “non-survival group.” We evaluated data on the patients’ background, survival, and differences in the serum markers associated with AE-IIPs and in oxygenation markers between the groups.ResultsAmong patient characteristics, median forced vital capacity was significantly higher in the survival group than in the non-survival group. Among serum markers, 7 days after the initiation of PMX-DHP therapy, serum lactate dehydrogenase (LDH) levels, white blood cell counts, and D-dimer levels were significantly higher and serum lymphocyte count was significantly lower in the non-survival group than in the survival group. PaO2/FiO2 ratio (P/F ratio) and alveolar-arterial oxygen difference (Aa-DO2) were significantly different between the two groups. The changes in LDH level (ΔLDH), P/F ratio, and Aa-DO2 were significantly different from day 1 to 7 days after the initiation of PMX-DHP between the two groups. Multivariate analysis revealed that ΔLDH was the only risk factor associated with poor prognosis. The cut-off value of ΔLDH was calculated as −8. The median survival time (MST) was significantly longer in patients with low ΔLDH values (<−8) than in those with high ΔLDH values (>−8).ConclusionsChanges in serum LDH levels are reasonable markers for evaluating the prognosis of PMX-DHP therapy.

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Section: Discussionsupporting

confidence: 50%

Serum Lactate Dehydrogenase Level As a Predictor Of Survival For Patients With Acute Exacerbation of Idiopathic Interstitial Pneumonias Undergoing Polymyxin B-Immobilized Fiber Column-Direct Hemoperfusion

Kato

Nakazawa

Akimoto

et al. 2021

Preprint

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“…[6][7][8] The studies mentioned above, however, did not use the current definition of AE-IPF in their inclusion criteria, and in addition, one study included patients treated in the ICU, 8 which is known to be a risk factor for death, at least in patients with IPF. 28 The other published studies concerning AE-FILD have been conducted in Japan, [9][10][11][12][13][14] of which only one demonstrated longer survival of patients with non-IPF compared with that of IPF 13 ; in another study, the prognosis of AE-IPF compared with other FILDs was even better. 9 Altogether, it can be stated that the earlier publications focusing on acute respiratory worsening or AE of IPF or non-IPF FILDs have consisted of small and heterogenic study populations with variable inclusion criteria, which complicate making comparisons between the investigations.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4] The phenomenon of acute exacerbation (AE) has been associated with high mortality in IPF, and AEs also seem to cause significant mortality in other FILDs. [5][6][7][8][9][10][11][12][13][14] According to previous studies, every year about 5%-15% of patients with IPF will experience an AE. 5 13 Recently, it was shown that the incidence of AE or death in a study consisting of various types of patients with FILD was 7.8% per year.…”

Section: Introductionmentioning

confidence: 99%

Prognosis and causes of death of patients with acute exacerbation of fibrosing interstitial lung diseases

Salonen¹,

Purokivi²,

Bloigu³

et al. 2020

BMJ Open Resp Res

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BackgroundThe aim of this study was to compare the clinical characteristics, causes of death and factors impacting on the prognosis of patients with idiopathic pulmonary fibrosis (IPF) and other fibrosing interstitial lung disease (FILD) with a history of acute exacerbation (AE) of IPF or FILD.MethodsRetrospective data of hospital treatment periods caused by AE-IPF and AE-FILD were collected from medical records. Clinical features and survival data of IPF and non-IPF cases were evaluated and compared. The underlying and immediate causes of death were gathered from death certificates.ResultsA total of 128 patients fulfilled the criteria for inclusion. IPF (n=79/62%), rheumatoid arthritis-associated interstitial lung disease (RA-ILD; n=17/14%) and asbestosis (n=11/8.6%) were the most common FILD subgroups in the study. The median survival after hospitalisation in AE-IPF was 2.6 months compared with 21 months in other AE-FILDs (p<0.001). The survival difference was not explained by age, gender or pulmonary function test results at the time of hospitalisation. Patients with non-specific interstitial pneumonia and RA-ILD had the most favourable prognosis. ILD was the most common underlying cause of death in both patients with IPF and with other FILD accounting for 87% and 78% of deaths, respectively.ConclusionsWe detected a significantly longer survival in AE of patients with non-IPF compared with that of AE-IPFs. The prognosis of patients was affected by the underlying lung disease since pulmonary fibrosis was the underlying cause of death in the majority of all patients with FILD having experienced an AE.

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“…The 2018 Japanese IPF treatment guidelines suggest that patients with AE-IPF should be treated with corticosteroids, including pulse therapy 15 . This is contradicted by the results of Mengshu et al, 16 who reported that high doses of corticosteroids are not bene cial for AE-ILD patients, and that the clinical outcomes of patients with AE-IPF mainly depend on the underlying clinical condition and the extent of lung injury from the AE. Thus, there remains a lack of consensus regarding proper treatment of AE-ILD.…”

Section: Discussionmentioning

confidence: 86%

Corticosteroid Responsiveness in Patients With Acute Exacerbation of Interstitial Lung Disease Admitted to the Emergency Department

Jang

Yong

Leem

et al. 2020

Preprint

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Interstitial lung disease (ILD), particularly idiopathic pulmonary fibrosis (IPF), has a poor prognosis. Corticosteroids are widely used in the treatment of acute exacerbation of ILD (AE-ILD). This study aimed to clarify the causes of AE-ILD, determine the efficacy of corticosteroids for treating AE-ILD, and detect differences in the mortality rate among subgroups of ILD. This was an observational retrospective single-center study. Patients with ILD who presented to the emergency department with acute respiratory symptoms from January 1, 2016, to December 31, 2018, were included. Patients with AE-ILD were classified into two groups depending on the prednisolone dose: low dose (0 to 1.0 mg/kg) or high dose (>1.0 mg/kg). Mortality rates between patients with and without IPF were compared. This study included 182 patients with AE-ILD, including IPF (n=117) and non-IPF (n=65). Multivariate Cox regression analysis showed that corticosteroid dose (HR:0.221, CI:0.102–0.408, P<0.001), initial P/F ratio (HR:0.995, CI:0.992–0.999, P=0.006), and mechanical ventilation within 3 days of hospitalization (HR:4.205, CI:2.059–8.589, P<0.001) were independent risk factors for mortality in patients with AE-ILD. This study showed that outcomes improve with higher doses of corticosteroids (>1 mg/kg prednisolone) in patients with AE-non-IPF-ILD. However, this was not the case in patients with AE-IPF.

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Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study

Cited by 43 publications

References 42 publications

Serum Lactate Dehydrogenase Level As a Predictor Of Survival For Patients With Acute Exacerbation of Idiopathic Interstitial Pneumonias Undergoing Polymyxin B-Immobilized Fiber Column-Direct Hemoperfusion

Serum Lactate Dehydrogenase Level As a Predictor Of Survival For Patients With Acute Exacerbation of Idiopathic Interstitial Pneumonias Undergoing Polymyxin B-Immobilized Fiber Column-Direct Hemoperfusion

Prognosis and causes of death of patients with acute exacerbation of fibrosing interstitial lung diseases

Corticosteroid Responsiveness in Patients With Acute Exacerbation of Interstitial Lung Disease Admitted to the Emergency Department

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