A cohort study of mortality predictors in patients with acute exacerbation of chronic fibrosing interstitial pneumonia

Usui, Yukio; Kaga, Akiko; Sakai, Fumikazu; Shiono, Ayako; Komiyama, Kenichiro; Hagiwara, Koichi; Kanazawa, Minoru

doi:10.1136/bmjopen-2013-002971

Cited by 45 publications

(42 citation statements)

References 35 publications

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“…In AE IPF, the cohort of 51 patients, as reported by Usui el al., the predictors of mortality were high systemic inflammatory response score and serum procalcitonin levels . However, in our study cohort, those who had significantly lower baseline PaO 2 had higher mortality indicating severe nature of the underlying connective tissue disease.…”

Section: Discussioncontrasting

confidence: 53%

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Clinical features and outcome of acute exacerbation in connective tissue disease‐associated interstitial lung disease: A single‐center study from India

et al. 2019

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Background Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is associated with high mortality, but there is limited clinical data on AE of interstitial lung disease (ILD) in connective tissue disease‐associated ILD (CTD ILD). The present study was conducted to provide prevalence and clinical features of AE, as well as various risk factors associated with mortality among AE CTD ILD patients. Methods Between May 2013 and April 2018, 15 patients who developed AE among 105 consecutive patients with CTD with chronic ILD were included. AE was defined using the criteria recently proposed by the IPF net, with slight modification for adaptation to CVD‐IP6 (collagen vascular disease‐associated interstitial pneumonia), and patients having CTD with AE met all the criteria. Results Fifteen patients with mean age of 45.8 ± 13.9 years developed AE; the most common subgroup (n = 5, 33%) was systemic sclerosis. The mean duration (months) between diagnosis of ILD and AE was 56.5 ± 38.0 with mean follow‐up duration of 24 ± 18.1 months. The baseline arterial oxygen pressure (PaO2) was 81.7 ± 8.1 mm Hg and mean forced vital capacity (%) was 57.9 ± 8.9. Five patients requiring mechanical ventilation died. Patients with shorter duration (months) of disease between onset of ILD to AE had higher mortality, 40.4 ± 45.1 vs 64.6 ± 33.6. Those who had significantly lower baseline PaO2 (mean ± SD), 72.6 ± 3.4 vs 86.2 ± 5.3 mm Hg (P = .002) had higher mortality. Conclusions In our study, the majority of patients with AE CTD ILD had systemic sclerosis. Patients with lower baseline PaO2 and those requiring mechanical ventilation had higher mortality.

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Section: Discussioncontrasting

confidence: 53%

“…In patients with AE, this was preceded by upper respiratory in- score and serum procalcitonin levels. 16 However, in our study cohort, those who had significantly lower baseline PaO 2 had higher mortality indicating severe nature of the underlying connective tissue disease.…”

Section: Discussionmentioning

confidence: 51%

Clinical features and outcome of acute exacerbation in connective tissue disease‐associated interstitial lung disease: A single‐center study from India

et al. 2019

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“…Higher composite HRCT scores including extent of ground-glass opacification, consolidation, traction bronchiectasis and honeycombing predict mortality [23,29]. Blood-based biomarkers of a worse outcome include elevated lactate dehydrogenase [23,41], C-reactive protein [6], Krebs von den Lungen-6 (KL-6) [29,41], pro-calcitonin [40], circulating fibrocytes [70], elevated interleukin-17 [28] and anti-heat shock protein 70 autoantibodies [39], but none of these have been validated prospectively, or impact clinical decision making. Recently, a staging system for AE with several variables was proposed [41].…”

Section: Influence Of Baseline Prognostic Variablesmentioning

confidence: 99%

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

2017

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Recognising recent advances, the definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) have been updated by an international working group. The new definition describes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterised by evidence of new widespread alveolar abnormality. The new criteria require a previous or concurrent diagnosis of IPF, an acute worsening or development of dyspnoea typically less than 1 month in duration, chest imaging evidence on computed tomography (CT) of new bilateral ground-glass opacity and/or consolidation superimposed on a background imaging pattern of usual interstitial pneumonia not fully explained by cardiac failure or fluid overload. Due to high in-hospital mortality rates, current treatment guidelines say that the majority of patients with AE-IPF should not receive mechanical ventilation. However, new data suggest that the prognosis may have improved. This modest improvement in overall survival seen in more recent studies may be the result of differences in the diagnostic criteria, study design, baseline clinical risk factors and/or improvements in management. Based on our updated knowledge of possible preventive and therapeutic measures, including mechanical ventilation and pharmacological therapies, the current approach to the treatment of AE-IPF requires careful decision-making.

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“…[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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A cohort study of mortality predictors in patients with acute exacerbation of chronic fibrosing interstitial pneumonia

Cited by 45 publications

References 35 publications

Clinical features and outcome of acute exacerbation in connective tissue disease‐associated interstitial lung disease: A single‐center study from India

Clinical features and outcome of acute exacerbation in connective tissue disease‐associated interstitial lung disease: A single‐center study from India

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

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

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