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

Salonen, Johanna; Purokivi, Minna; Bloigu, Risto; Kaarteenaho, Riitta

doi:10.1136/bmjresp-2020-000563

Cited by 21 publications

(29 citation statements)

References 46 publications

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“…Moua et al have observed that in non-IPF patients, an AE-ILD did not cause increased mortality in comparison with other types of hospitalizations in a multivariate model [5]. These ndings may re ect the fact that AE-ILDs are less fatal for non-IPF patients than for those patients with IPF, which was also supported by our previous study, where IPF patients had a signi cantly shorter survival after an AE in comparison with non-IPF patients [14].…”

Section: Discussionsupporting

confidence: 82%

“…The study material consists of ILD patients who were non-electively hospitalized due to acute respiratory symptoms in OUH or OH between 1/1/2008 and 31/12/2017. A total of 128 of these patients were also included in our previous study [14]. The owchart of the study and criteria used to de ne AE-ILD are presented in Fig.…”

Section: Patient and Data Collectionmentioning

confidence: 99%

“…1. We performed a search with International Classi cation of Diseases version 10 (ICD-10) codes J84.1, J84.8 and J84.9 to identify the patients and their treatment periods from medical records as described previously [14]. Another search was performed with ICD-10 codes J61, J99, J99.0* and J99*M05.1 to nd the hospitalizations related to asbestosis and rheumatoid arthritis-associated ILD (RA-ILD).…”

Section: Patient and Data Collectionmentioning

confidence: 99%

“…The clinical information was collected systematically from electronic patient records dating back about 20 years in OUH and OH in a form specially designed for the present study. A detailed description of the data collection and evaluation of ILD types has been provided in our previous study [14].…”

Section: Patient and Data Collectionmentioning

confidence: 99%

“…The data on hospitalizations was collected related to the rst non-elective hospitalization after or at the time of ILD diagnosis, if the diagnosis of ILD was made during a non-elective hospitalization. The information on the patients having experienced an AE-ILD after their rst respiratory hospitalization was gathered as described previously [14]. Thus, we had the data of both the patients with AE-ILD as the cause for the rst non-elective hospital treatment period and the data on those patients who were rst hospitalized due to some other reason but experienced an AE-ILD later during the follow-up.…”

Section: Patient and Data Collectionmentioning

confidence: 99%

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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: Discussionsupporting

confidence: 82%

Section: Patient and Data Collectionmentioning

confidence: 99%

Section: Patient and Data Collectionmentioning

confidence: 99%

Section: Patient and Data Collectionmentioning

confidence: 99%

Section: Patient and Data Collectionmentioning

confidence: 99%

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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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Interstitial Lung Disease

Maher

2024

JAMA

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ImportanceInterstitial lung disease (ILD) consists of a group of pulmonary disorders characterized by inflammation and/or fibrosis of the lung parenchyma associated with progressive dyspnea that frequently results in end-stage respiratory failure. In the US, ILD affects approximately 650 000 people and causes approximately 25 000 to 30 000 deaths per year.ObservationsThe most common forms of ILD are idiopathic pulmonary fibrosis (IPF), which accounts for approximately one-third of all cases of ILD, hypersensitivity pneumonitis, accounting for 15% of ILD cases, and connective tissue disease (CTD), accounting for 25% of ILD cases. ILD typically presents with dyspnea on exertion. Approximately 30% of patients with ILD report cough. Thoracic computed tomography is approximately 91% sensitive and 71% specific for diagnosing subtypes of ILDs such as IPF. Physiologic assessment provides important prognostic information. A 5% decline in forced vital capacity (FVC) over 12 months is associated with an approximately 2-fold increase in mortality compared with no change in FVC. Antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% in individuals with IPF, scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause. For connective tissue disease–associated ILD, immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up. Structured exercise therapy reduces symptoms and improves 6-minute walk test distance in individuals with dyspnea. Oxygen reduces symptoms and improves quality of life in individuals with ILD who desaturate below 88% on a 6-minute walk test. Lung transplant may improve symptoms and resolve respiratory failure in patients with end-stage ILD. After lung transplant, patients with ILD have a median survival of 5.2 to 6.7 years compared with a median survival of less than 2 years in patients with advanced ILD who do not undergo lung transplant. Up to 85% of individuals with end-stage fibrotic ILD develop pulmonary hypertension. In these patients, treatment with inhaled treprostinil improves walking distance and respiratory symptoms.Conclusions and RelevanceInterstitial lung disease typically presents with dyspnea on exertion and can progress to respiratory failure. First-line therapy includes nintedanib or pirfenidone for IPF and mycophenolate mofetil for ILD due to connective tissue disease. Lung transplant should be considered for patients with advanced ILD. In patients with ILD, exercise training improves 6-minute walk test distance and quality of life.

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Nintedanib in Patients With Autoimmune Disease–Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial

Matteson

Kelly

Distler

et al. 2022

Arthritis & Rheumatology

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OBJECTIVE: To analyze the efficacy and safety of nintedanib in patients with fibrosing autoimmune disease-related interstitial lung diseases (ILDs) with a progressive phenotype. METHODS: The INBUILD trial enrolled subjects with a fibrosing ILD other than idiopathic pulmonary fibrosis, with diffuse fibrosing lung disease of >10% extent on high-resolution computed tomography, forced vital capacity (FVC) 45% predicted and diffusing capacity of the lungs for carbon monoxide 30%-<80% predicted. Subjects fulfilled protocol-defined criteria for progression of ILD within the 24 months before screening, despite management deemed appropriate in clinical practice. Subjects were randomized to receive nintedanib or placebo. We assessed the rate of decline in FVC (mL/year) and adverse events over 52 weeks in the subgroup with autoimmune disease-related ILDs. RESULTS: Among 170 subjects with autoimmune disease-related ILDs, the rate of decline in FVC over 52 weeks was -75.9 mL/year with nintedanib versus -178.6 mL/year with placebo (difference 102.7 mL/year [95% CI 23.2, 182.2]; nominal P=0.012). No heterogeneity was detected in the effect of nintedanib versus placebo across subgroups by ILD diagnosis (P=0.91). The most frequent adverse event was diarrhea, reported in 63.4% and 27.3% of subjects in the nintedanib and placebo groups, respectively. Adverse events led to permanent discontinuation of trial drug in 17.1% and 10.2% of subjects in the nintedanib and placebo groups, respectively. CONCLUSION: In the INBUILD trial, nintedanib slowed the rate of decline in FVC in subjects with progressive fibrosing autoimmune disease-related ILDs, with adverse events that were manageable for most subjects.

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

Cited by 21 publications

References 46 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

Interstitial Lung Disease

Nintedanib in Patients With Autoimmune Disease–Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial

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