Acute exacerbations of fibrotic interstitial lung diseases

Suzuki, Atsushi; Kondoh, Yasuhiro; Brown, Kevin K.; Johkoh, Takeshi; Kataoka, Kensuke; Fukuoka, Junya; Kimura, Tomoki; Matsuda, Toshiaki; Yokoyama, Toshiki; Fukihara, Jun; Ando, Masahiko; Tanaka, Tomonori; Hashimoto, Naozumi; Sakamoto, Koji; Hasegawa, Yoshinori

doi:10.1111/resp.13682

Cited by 115 publications

(114 citation statements)

References 40 publications

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“…In our study, the GAP index was a risk factor for both death and AE-ILD. At least one of these ndings has been described in earlier investigations [18,[22][23][24][25][26][27], even though this has not been veri ed in all studies [9,28]. We also demonstrated that AE-ILDs can occur at any phase of the disease in a cohort involving both IPF and non-IPF patients, a nding which is a known feature of IPF, making the disease course di cult to predict [12,29].…”

Section: Discussionmentioning

confidence: 56%

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Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Salonen

Vähänikkilä

Purokivi

et al. 2020

Preprint

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Background Acute exacerbation of ILD (AE-ILD) is a common reason for hospitalization; it is also associated with significant mortality. Less is known about the prognostic significance of other events causing acute, non-elective hospitalizations in ILD patients. Methods ILD patients hospitalized due to acute respiratory worsening were collected from medical records. Reasons for respiratory deterioration were classified into AE-ILDs and other causes. Clinical features and survival data of idiopathic pulmonary fibrosis (IPF) and other types of ILDs were evaluated and compared. Results In all, 237 patients (138 with IPF and 99 with other ILD) fulfilled the inclusion criteria. Of the non-IPF ILD types, the most prevalent subgroups were connective tissue disease-associated ILD (n = 33) and asbestosis (n = 22). The most common cause for hospitalization was AE-ILD explaining 41% of hospitalizations. Lower respiratory tract infection (22%), subacute progression of ILD (12%) and cardiovascular causes (7.2%) were other common reasons for hospital treatment. Patients with a lower respiratory tract infection had a more favorable prognosis compared with patients with AE-ILD. AE-ILDs were less fatal than cardiovascular or concurrent non-ILD-related causes for hospitalizations in non-IPF patients. High Gender-Age-Physiology (GAP) index was a marker for shortened survival and earlier AE-ILDs in all patients. IPF patients had a significantly shorter overall and post-hospitalization survival time compared with other ILDs. Conclusions Most respiratory hospitalizations in ILD patients were related to causes other than AE-ILD, which highlights the importance of accurate differential diagnosis in order to target the appropriate treatment for each ILD patient.

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Section: Discussionmentioning

confidence: 56%

“…54%, in our study population. In the previous studies, presenting data on GAP and its effect on mortality, the prevalence of AE-ILD has varied between 20-40% [18,22,23], these discrepancies may be caused by different types of study protocols, e.g. we included only non-electively hospitalized patients.…”

Section: Discussionmentioning

confidence: 99%

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Salonen

Vähänikkilä

Purokivi

et al. 2020

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: 98%

“…[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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“…Patients with AE of IPF were eligible for the review. Idiopathic NSIP was not included in this study because there has been no clear evidence that the prognosis of AE of idiopathic NSIP is the same as that of AE of IPF although it constitutes CFIP alongside of IPF [19]. IPF was diagnosed based on previously published joint statement of American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association (ATS/ERS/JRS/ALAT) or its recent update [10,20].…”

Section: Eligibility Criteria Subjectsmentioning

confidence: 99%

The efficacy of recombinant human soluble thrombomodulin (rhsTM) treatment for acute exacerbation of idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Kamiya

Panlaqui

2020

BMC Pulm Med

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Background: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is devastating with no established treatment. This phenomenon involves disordered coagulation and excessive inflammatory reactions. As recombinant human soluble thrombomodulin (rhsTM) possesses anti-coagulative and anti-inflammatory properties, the medicine is expected to improve the prognosis of the disease. The aim of this study was to summarize current evidence regarding benefits and harms of rhsTM treatment for AE of IPF. Method: Patients with AE of IPF were eligible for the review and all of the other types of interstitial pneumonias were excluded. The effect of rhsTM treatment on the outcomes such as all-cause mortality was estimated in comparison to conventional therapy. Primary studies of any design aside from a case report were reviewed. Electronic databases such as Medline and EMBASE were searched from 2002 through August 14, 2019. Two reviewers independently selected eligible reports and extracted relevant data. A risk of bias of individual studies was assessed similarly. Meta-analysis was conducted for univariate results if at least three studies were available for the same outcome. Result: Out of a total of 390 records identified, eight studies were first deemed eligible and four of them were finally focused for the review. Only one study was a prospective trial and a historical control was employed in all studies. An overall risk of bias was rated as serious in three out of four studies. A total of 169 subjects were included. Two out of three studies that reported 3-month all-cause mortality by univariate analysis demonstrated beneficial effects of rhsTM treatment and a pooled analysis demonstrated that rhsTM treatment improved 3-month all-cause mortality with a risk ratio of 0.50 (95% confidence interval (CI): 0.35-0.72). All two studies reporting multivariate results demonstrated that rhsTM treatment improved 3-month all-cause mortality with odds ratios of 0.21 (95% CI: 0.05-0.91) and 0.25 (95% CI: 0.09-0.68), respectively. There were no serious adverse events. Conclusion: The rhsTM treatment was demonstrated to improve 3-month all-cause mortality of AE of IPF with no serious adverse events. However, these findings should be interpreted with caution due to a small number of studies and serious risk of bias.

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Acute exacerbations of fibrotic interstitial lung diseases

Cited by 115 publications

References 40 publications

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

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

The efficacy of recombinant human soluble thrombomodulin (rhsTM) treatment for acute exacerbation of idiopathic pulmonary fibrosis: a systematic review and meta-analysis

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