Diseases

Progressive Pulmonary Fibrosis

Progressive pulmonary fibrosis (PPF) is an umbrella term that encompasses a group of interstitial lung diseases characterized by progressive, self-sustaining pulmonary fibrosis (damage and scarring of lung tissue), both from known causes (other underlying diseases) and for unknown reasons. PPF shows a disease course similar to idiopathic pulmonary fibrosis (IPF), which clinical guidelines classify as a distinct entity from the rest of the diseases that make up PPF.

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PPF can develop in idiopathic interstitial pneumonias other than IPF, autoimmune ILD, fibrotic ILD related to environmental or occupational exposure, and chronic fibrosing hypersensitivity pneumonitis (chronic scarring of the lungs from allergies). It is estimated that between 10.4% and 60.6% of patients with these diseases will develop PPF¹.

Patients with PPF share symptoms with IPF. The most common symptoms are shortness of breath, coughing, and fatigue, which can limit patients' ability to perform physical tasks and participate in social activities. This causes low mood and isolation, and makes it difficult to perform daily activities.

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PPF has a significant impact on patients' quality of life, affecting their daily lives, psychological well-being, professional career, and personal relationships. Patients with PPF have decreased lung function and increased mortality despite usual treatments for underlying ILD. The median transplant-free survival and overall survival are estimated to be approximately 2.9 years and 3.7 years, respectively^1,2.

How quickly pulmonary fibrosis gets worse over time and the severity of symptoms can vary greatly from person to person. Some people get very sick very quickly, others have moderate symptoms that get worse more slowly, over months or years.

Cottin V, Teague R, Nicholson L, Langham S, Baldwin M. The Burden of Progressive-Fibrosing Interstitial Lung Diseases. Front Med (Lausanne). 2022 Feb 1; 9:7999
Platenburg MGJP, van der Vis JJ, Grutters JC, van Moorsel CHM. Decreased survival and lung function in progressive pulmonary fibrosis. Medicine. 2023;59:296.