Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

Abstract

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial

“…However, ILD is characterised by more frequent and severe exercise-induced desaturation (6) compared to COPD, indicating that ILDspecific studies are needed (7) . The paucity of data on ambulatory oxygen in ILD is reflected by a lack of guidance in national and international guidelines (8)(9)(10).…”

The supportive care needs of people living with pulmonary fibrosis and their caregivers: a systematic review

“…Therefore, it seems likely that advances in supportive care have not kept pace with advances in other aspects of treatment. For instance, while anxiety and depression have been thoroughly documented in people with pulmonary fibrosis in recent years, specific treatment strategies for this group are lacking [43]. The enormous psychosocial and emotional burden of pulmonary fibrosis documented in this review suggests that this should be a priority for clinical practice and future research.…”

Putting lung function reference equations into context

“…A 69-year-old man with idiopathic pulmonary fibrosis (IPF) is undertaking routine lung function testing prior to clinical review and assessment for consideration of antifibrotics. As he lives in Australia, the pulmonary function eligibility criteria for antifibrotics are forced vital capacity (FVC) ≥50% of predicted and transfer factor of the lung for carbon monoxide ( T LCO ) ≥30% of predicted in the absence of obstruction [ 1 ].…”