Acute exacerbations of progressive-fibrosing interstitial lung diseases

Kolb, Martin; Bondue, Benjamin; Pesci, Alberto; Miyazaki, Yasunari; Song, Jin Woo; Bhatt, Nitin; Huggins, John; Oldham, J.; Padilla, María; Roman, Jesse; Shapera, Shane

doi:10.1183/16000617.0071-2018

Cited by 118 publications

(94 citation statements)

References 91 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…pneumothorax, pleural effusion or cardiac overload) [45]. Kolb et al [46] provide a detailed discussion of acute exacerbations. Acute exacerbations occur infrequently, with typical annual incidences between 5% and 15% in IPF [47].…”

Section: Acute Exacerbationmentioning

confidence: 99%

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

et al. 2018

View full text Add to dashboard Cite

There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This review discusses the burden of ILDs that may present a progressive fibrosing phenotype and the factors impacting healthcare utilisation.

show abstract

Section: Acute Exacerbationmentioning

confidence: 99%

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

et al. 2018

View full text Add to dashboard Cite

show abstract

“…5 17 It has been proposed that it would be beneficial to apply the definition of AE-IPF also to non-IPF FILDs. 16 Currently, AE-IPFs are divided into triggered and idiopathic exacerbations, and the exclusion of infection by bronchoalveolar lavage or endotracheal aspirate is no longer a requirement as was the case in the older recommendations. Thus, a significant number of cases that would fulfil the present criteria for triggered AEs have been excluded from earlier research materials.…”

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

View full text Add to dashboard Cite

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.

show abstract

“…Table 1 presents data from previously published epidemiological studies and real-world data (RWD) studies on hospitalizations of ILD patients [5][6][7][8][9][10][11]. Table 1 here Acute exacerbation (AE) is a severe complication of ILD, which seems to cause signi cant mortality in all types of ILDs [12,13]. The current diagnostic criteria for AE-IPF have been suggested to be applied for other ILDs as well [12,13].…”

Section: Introductionmentioning

confidence: 99%

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

Salonen

Vähänikkilä

Purokivi

et al. 2020

Preprint

View full text Add to dashboard Cite

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.

show abstract

Acute exacerbations of progressive-fibrosing interstitial lung diseases

Cited by 118 publications

References 91 publications

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

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

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

Contact Info

Product

Resources

About