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

Abstract

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.

Pulmonary artery enlargement predicts poor outcome during acute exacerbations of fibrotic interstitial lung disease

“…However, we did not find a difference in outcome between the patients treated in 2009 and those in 2016. Indeed, there are still no proven beneficial management strategies for AE of f‐ILD and the therapeutic strategies were similar throughout the study period. One change was the change of diagnostic criteria in 2016, which AE‐IPF proposed to include patients with triggered AE as well as idiopathic AE .…”

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

“…Previously, the in-hospital mortality of AE-IPF was reported to be over 50%, and in the patients on mechanical ventilation, the in-hospital mortality is 87% with an overall mortality of 94% among 135 cases reported (Mallick, 2008). However, more recent studies suggest a slightly better survival, with 1 month-survival rate of 66% (range: 47-85%), 3 month rate of 41% (range: 0-54%), and survival to hospital discharge of 44% (4-77%) (Kondoh et al, 2017). For patients treated with IMV or NIV, in-hospital survival rates have been reported to be 49.4% with IMV and 69.1% with NIV, in the United States (Rush et al, 2016) and a 30 day-survival rate of 44.6% and 3 month-survival rate of 24.6% in Japan (Oda et al, 2016).…”

Therapie von Komplikationen und nichtpharmakologisches Management der idiopathischen pulmonalen Fibrose

“…Prognose. In den ältesten Studien wurde eine Krankenhausletalität bis 90 % beschrieben [21], während die neuesten über eine 90-Tage Mortalität zwischen 40 und 60 % berichten [22]. Unterschiede in den diagnostischen Kriterien und ihrer Anwendung,die frühzeitige Therapieeinleitung sowie ein besseres Management inklusive akkurater Selektion der Patienten zur invasiven/nichtinvasiven Ventilation können mit der Verbesserung des Überlebens assoziiert sein [22].…”

“…Die meisten Daten beruhen auf kleinen Kohortenstudien und Befragungen [22]. Standardisierte Therapien liegen nicht vor, das gilt auch für die häufig eingesetzte systemische Kortikosteroidtherapie.…”