Diseases

Progressive Pulmonary Fibrosis

Progressive pulmonary fibrosis (PPF) is a general term that encompasses a group of interstitial lung diseases characterized by progressive and self-sustaining pulmonary fibrosis (damage and scarring of lung tissue), whether due to known causes (other underlying diseases) or for unknown reasons. PPF exhibits a disease course similar to that of idiopathic pulmonary fibrosis (IPF), which clinical guidelines classify as a distinct entity from the other diseases that make up PPF¹.

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PPF may develop in idiopathic interstitial pneumonias other than IPF, autoimmune ILDs, fibrotic ILDs related to environmental or occupational exposure, and chronic hypersensitivity pneumonitis (chronic scarring of the lungs due to allergies). It is estimated that between 10.4% and 60.6% of patients with these diseases will develop PPF².

Patients with PPF share symptoms with IPF. The most frequent symptoms are shortness of breath, cough, and fatigue, which may limit patients’ ability to perform physical tasks and participate in social activities. This leads to low mood and social isolation, and makes daily activities more difficult to carry out².

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PPF significantly affects patients' quality of life, impacting daily activities, psychological well-being, professional life, and personal relationships. Patients with PPF have reduced lung function and increased mortality despite usual treatments for the underlying ILD. Median transplant-free survival and overall survival are estimated at approximately 2.9 and 3.7 years, respectively^2,3. The speed at which fibrosis progresses and symptom severity vary widely among individuals. Some become severely ill very rapidly, whereas others experience moderate symptoms that worsen more slowly over months or years.

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.

Raghu G, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e19–e47.
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. Medicina. 2023;59:296.