Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia

Arai, Toru; Kagawa, Tomoko; Sasaki, Yumiko; Sugawara, Reiko; Sugimoto, Chikatoshi; Tachibana, Kazunobu; Kitaichi, Masanori; Akira, Masanori; Hayashi, Seiji; Inoue, Yoshikazu

doi:10.1111/resp.12862

Cited by 44 publications

(52 citation statements)

References 29 publications

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“…In general, fibrotic NSIP, CHP, CTD‐ILD and UC‐ILD are the most frequent diagnoses in the differential with IPF. Previously published data showed that the frequencies of AE in these categories were lower than that of AE‐IPF . In our study, there was no significant difference in the frequency and short‐term outcomes of AE among FILD subtypes.…”

Section: Discussioncontrasting

confidence: 59%

“…This group includes the fibrotic form of non‐specific interstitial pneumonia (NSIP), chronic (fibrotic) hypersensitivity pneumonitis (CHP), connective tissue disease‐associated ILD (CTD‐ILD), unclassifiable ILD (UC‐ILD) and others. Currently, there are limited data regarding the frequency and prognosis of AE in patients with FILD . The aim of this study is to compare the incidence, risk factors and outcomes between AE in IPF and non‐IPF forms of FILD.…”

Section: Introductionmentioning

confidence: 99%

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Acute exacerbations of fibrotic interstitial lung diseases

et al. 2019

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Background and objective: Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE-IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD). Methods: We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (n = 462) and other FILD which included non-specific interstitial pneumonia (n = 22), chronic hypersensitivity pneumonitis (n = 29), connective tissue disease-associated ILD (n = 205) and unclassifiable ILD (n = 209). Using the 2016 definition of AE-IPF, we identified all subjects with an AE. Results: During the observational period, 193 patients experienced a first AE (AE-FILD n = 69, AE-IPF n = 124). The time to first AE was significantly longer in FILD than IPF (log-rank test, P < 0.001). After adjusting for potentially influential confounders, FILD remained a significant predictor of longer time to first AE compared with IPF (hazard ratio: 0.453; 95% CI: 0.317-0.647, P = 0.006). In a multivariate Cox proportional analysis, baseline disease severity was closely associated with the incidence of AE-ILD. Even after adjustment for other clinical variables, AE had a negative impact on overall survival. AE-FILD and AE-IPF showed similar poor short-term outcomes. Conclusion: All forms of ILD are at risk of AE and have a similar outcome to AE-IPF. SUMMARY AT A GLANCEThis study reveals that all forms of fibrotic interstitial lung diseases (FILD) are at risk of acute exacerbations (AE) and have similar outcomes to AE of idiopathic pulmonary fibrosis (AE-IPF). Clinicians are recommended to consider all forms of FILD to be at risk of AE.

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Section: Discussioncontrasting

confidence: 59%

Section: Introductionmentioning

confidence: 99%

Acute exacerbations of fibrotic interstitial lung diseases

et al. 2019

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“…Especially in the fibrotic ILDs, AE accounts for significant morbidity and mortality. [80][81][82] Although no consensus definition for AE-ILD exists, the generally accepted criteria are extrapolated from the IPF population given the similarities in presentation. AE is generally characterized by acutely (typically < 1 month) worsening dyspnea and parenchymal infiltrates on imaging.…”

Section: Acute Exacerbation In Ildsmentioning

confidence: 99%

The Role of Infection in Interstitial Lung Diseases

Azadeh

Limper

Carmona

et al. 2017

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Interstitial lung disease (ILD) comprises an array of heterogeneous parenchymal lung diseases that are associated with a spectrum of pathologic, radiologic, and clinical manifestations. There are ILDs with known causes and those that are idiopathic, making treatment strategies challenging. Prognosis can vary according to the type of ILD, but many exhibit gradual progression with an unpredictable clinical course in individual patients, as seen in idiopathic pulmonary fibrosis and the phenomenon of "acute exacerbation"(AE). Given the often poor prognosis of these patients, the search for a reversible cause of respiratory worsening remains paramount. Infections have been theorized to play a role in ILDs, both in the pathogenesis of ILD and as potential triggers of AE. Research efforts thus far have shown the highest association with viral pathogens; however, fungal and bacterial organisms have also been implicated. This review aims to summarize the current knowledge on the role of infections in the setting of ILD.

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“…have been recently updated. AE is the most common immediate cause of death in IPF, and improved management of AE‐IPF purportedly leads to a better disease prognosis. In addition, AE reportedly occurs in patients with idiopathic interstitial pneumonias (IIP) other than IPF (non‐IPF) …”

Section: Introductionmentioning

confidence: 99%

Recombinant thrombomodulin for acute exacerbation in idiopathic interstitial pneumonias

et al. 2019

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Background and objective: Acute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) or other idiopathic interstitial pneumonias (IIP) is a poor prognostic event despite conventional therapy with corticosteroids and/or immunosuppressants. We aimed to evaluate the efficacy and safety of recombinant human soluble thrombomodulin (rhTM) for AE-IIP. Methods: For this prospective single-arm open-label multicentre cohort study, we retrospectively registered 61 cases of AE-IIP treated with conventional therapy between 2011 and 2013 (control arm), and prospectively enrolled 39 cases of AE-IIP treated with conventional therapy and rhTM (380 U/kg/day for 6 days) between 2014 and 2016 (rhTM arm). To reduce potential confounding in treatment comparisons, an adjusted mortality analysis for 90-day survival was conducted with weighted Cox proportional hazards regression models using inverse probability of treatment weighting. Weights were derived from propensity scores estimated using a multivariable logistic regression analysis including potential confounders. Results: The 90-day survival rates of AE-IIP patients treated with/without rhTM were 66.7% (26/39) and 47.5% (29/61), respectively. After adjusting for imbalances, rhTM therapy was significantly associated with reduced mortality (adjusted hazard ratio (HR): 0.453; 95% CI: 0.237-0.864; P = 0.0163). The frequencies of adverse events with/without rhTM were 17.9% (7/39) and 19.7% (12/61), which were similar in both arms (P = 1.0). Two bleeding-related adverse events occurred in the rhTM arm. Conclusion: Safety and efficacy were observed for rhTM treatment of AE-IIP. A future randomized controlled trial is required to draw final conclusions.Clinical trial registration: UMIN000014969 at www.umin. ac.jp

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Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia

Cited by 44 publications

References 29 publications

Acute exacerbations of fibrotic interstitial lung diseases

Acute exacerbations of fibrotic interstitial lung diseases

The Role of Infection in Interstitial Lung Diseases

Recombinant thrombomodulin for acute exacerbation in idiopathic interstitial pneumonias

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