Progressive pulmonary fibrosis: an expert group consensus statement

Abstract

This expert group consensus statement emphasises the need for standardising the definition of progressive fibrosing interstitial lung diseases (F-ILDs), with an accurate initial diagnosis being of paramount importance in ensuring appropriate initial management. Equally, case-by-case decisions on monitoring and management are essential, given the varying presentations of F-ILDs and the varying rates of progression. The value of diagnostic tests in risk stratification at presentation and, separately, the importance of a logical monitoring strategy, tailored to manage the risk of progression, are also stressed. The term “progressive pulmonary fibrosis” (PPF) exactly describes the entity that clinicians often face in practice. The importance of using antifibrotic therapy early in PPF (once initial management has failed to prevent progression) is increasingly supported by evidence. Artificial intelligence software for high-resolution computed tomography analysis, although an exciting tool for the future, awaits validation. Guidance is provided on pulmonary rehabilitation, oxygen and the use of non-invasive ventilation focused specifically on the needs of ILD patients with progressive disease. PPF should be differentiated from acute deterioration due to drug-induced lung toxicity or other forms of acute exacerbations. Referral criteria for a lung transplant are discussed and applied to patient needs in severe diseases where transplantation is not realistic, either due to access limitations or transplantation contraindications. In conclusion, expert group consensus guidance is provided on the diagnosis, treatment and monitoring of F-ILDs with specific focus on the recognition of PPF and the management of pulmonary fibrosis progressing despite initial management.

Effect of Ambulatory Oxygen on the Respiratory Pattern during the 6 Min Walking Test in Patients with Interstitial Lung Diseases

“…Indeed, in our patients, mean walked distance expressed as percent of predicted changed from 73% (64-83) on placebo to 79% (71-87) on oxygen, thus remaining on average below the normal value. However, while pharmacological treatments are available to slow disease progression in these patients [16], no treatment is available to them to improve exercise tolerance, except ambulatory oxygen and pulmonary rehabilitation [17], which are not mutually exclusive and could be combined [18], so even a partial result is clinically relevant. Due to the requirements of our experimental settings, we had to use a walking circuit of 20 m, slightly shorter than the recommended length of 30 m [14].…”

“…Not only is ambulatory oxygen generally underprescribed in interstitial lung diseases [3], and only recently found its way on expert recommendations [17], but often patients are reluctant to accept and use it [23], partly because it is difficult for them to understand its purpose and possible benefits. Our data, obtained in a familiar context such as walking, could be helpful to communicate to patients the expected benefits, in a setting of shared decision making [24].…”

Interstitial Lung Disease Associated with Systemic Sclerosis

“…When initiating nintedanib in SSc-ILD, a benefit of maintaining prolonged therapy with immunosuppressants in combination with nintedanib is less likely in the absence of previous response to immunosuppressants, and in case of recurrent infections. 178 Upfront combination therapy is generally reserved for patients with severe ILD. High-dose glucocorticoids (> 15 mg per day of prednisone) should be avoided.…”

Progressive Pulmonary Fibrosis: Where Are We Now?

“…Fibrosing ILD refers to a group of lung disorders characterized by the presence of fibrosis or scarring within the lung parenchyma [ 9 ]. The diagnosis typically involves a combination of clinical evaluation, imaging tests, pulmonary function tests, and occasionally a lung biopsy [ 7 ].…”