Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World

Abstract

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication associated with a high mortality. However, evidence and guidance on management is sparse. The aim of this international survey was to assess differences in prevention, diagnostic and treatment strategies for AE-IPF in specialised and non-specialised ILD centres worldwide.Material and Methods: Pulmonologists working in specialised and non-specialised ILD centres were invited to participate in a survey designed by an international expert panel. Responses were evaluated in respect to the physicians' institutions.Results: Three hundred and two (65%) of the respondents worked in a specialised ILD centre, 134 (29%) in a non-specialised pulmonology centre. Similarities were frequent with regards to diagnostic methods including radiology and screening for infection, treatment with corticosteroids, use of high-flow oxygen and non-invasive ventilation in critical ill patients and palliative strategies. However, differences were significant in terms of the use of KL-6 and pathogen testing in urine, treatments with cyclosporine and recombinant thrombomodulin, extracorporeal membrane oxygenation in critical ill patients as well as antacid medication and anaesthesia measures as preventive methods.Conclusion: Despite the absence of recommendations, approaches to the prevention, diagnosis and treatment of AE-IPF are comparable in specialised and non-specialised ILD centres, yet certain differences in the managements of AE-IPF exist. Clinical trials and guidelines are needed to improve patient care and prognosis in AE-IPF.

Comparing outcomes of ILD patients managed in specialised versus non-specialised centres

“…Furthermore, inhaled treprostinil recently achieved positive effects on outcomes [ 37 ]. A recent international survey could not reveal significant management differences regarding acute exacerbations—an often lethal complication of fibrosing ILDs—between specialised ILD centres and non-specialised centres [ 38 ]. This might explain why our data do not suggest that management at specialised ILD centres has an impact on patterns of respiratory-related hospitalisation.…”

IPF-Acute Exacerbations: Advances and Future Perspectives

“…Thirteen years later, in an international expert’s report on IPF, the idiopathic nature of IPF-AEs constitutes the first hypothesis on their etiology, though the dilemma “do acute exacerbations of IPF represent a distinct pathobiological manifestation of the primary disease process or are they caused by occult complications such as infection and aspiration” dominates the key questions remaining for future research ( Collard et al, 2007 ). In the same document, it is recognized that “diffuse alveolar damage superimposed on underlying UIP is the most commonly described finding when surgical lung biopsy is performed in patients with AE-IPF” ( Kondoh et al, 1993 ; Ambrosini et al, 2003 ; Rice et al, 2003 ; Parambil et al, 2005 ; Kim et al, 2006 ), although “organizing pneumonia without other evidence of organizing diffuse alveolar damage and extensive fibroblastic foci” has also been described in a few cases ( Churg et al, 2007 ) and that “treatment of IPF-AEs has generally consisted of high-dose corticosteroids, without any data from controlled trials to prove their efficacy.” Admission in some cases equals permission for most, and the aforementioned therapeutic approach represents the standard of care to date all over the world in both specialized and non-specialized ILD centers ( Polke et al, 2021 ). However, in most centers, broad-spectrum antibiotics plus macrolides were also administered and in some of them, immunosuppressants too were administered (cyclosporine A, intravenous cyclophosphamide, or tacrolimus) ( Polke et al, 2021 ).…”

“…The evidence regards mostly stable IPF ( Martinez et al, 2021 ), whereas randomized, controlled trials examining the role of antimicrobials in the management of IPF-AEs are still lacking. Furthermore, the role of antimicrobials is scarcely mentioned or analyzed in trials examining the immunosuppressive treatment in IPF-AE, in addition to their widespread use in everyday clinical practice ( Polke et al, 2021 ). In a retrospective study by Oda et al, it was shown that the administration of co-trimoxazole and macrolides was significantly associated with good prognosis in ventilated patients with IPF showing a rapid progression of respiratory failure and being treated with high doses of steroids ( Oda et al, 2016 ).…”

“…In conclusion, for some, controversy still surrounds the treatment of IPF-AEs, but not for all ( Papiris et al, 2014 ). “Well and appropriately designed studies” are needed, are ethical, or the time has come to attain a decision against the “paradox” of the common practice in IPF-AEs ( Polke et al, 2021 ), long awaited and owed to our patients.…”

Federal clinical guidelines on diagnosis and treatment of idiopathic pulmonary fibrosis

“…Методы терапии обострения ИЛФ с доказанной эффективностью, включая ГКС, отсутствуют. В связи с этим в настоящее время невозможно представить специфические рекомендации о дозах, путях введения и длительности терапии ГКС либо другими лекарственными препаратами при обострении ИЛФ [69,[88][89][90].…”