What is PF/ILD: Disease pathways and treatment

This resource, research and latest developments: what’s on the horizon for pulmonary fibrosis, is a recording of the patient session presented at the Australasian Rare Lung Disease Conference. Pulmonary fibrosis (PF), including idiopathic pulmonary fibrosis (IPF), involves abnormal lung repair leading to scarring. Unlike idiopathic pulmonary fibrosis, other types of pulmonary fibrosis have different causes, prognoses, and treatments. It’s crucial to accurately diagnose the specific type of pulmonary fibrosis, as treatments vary. Risk factors include pollution, smoking, and possibly reflux. In IPF, repeated lung injury and defective repair cause scarring, making the lungs stiff and less efficient at oxygen transfer. Diagnosing pulmonary fibrosis involves various tests, including scans and blood tests. Advances in radiology have significantly improved the definition and diagnosis of these conditions.

Patients with pulmonary fibrosis often experience breathlessness, cough, fatigue, and weight loss. While the average survival rate for untreated patients is 2-4 years, current holistic clinical services and treatments are improving outcomes. Exercise is crucial at any stage of respiratory illness, benefiting muscle, cardiac, bone, joint, and mental health. Preventing infections through vaccination and maintaining a healthy weight and diet are also important. Supportive and palliative care, which focuses on improving quality of life for those with chronic illnesses, is essential. Antifibrotic therapies, such as nintedanib and pirfenidone, help slow disease progression and improve patient outcomes, significantly improving survival rates for patients with pulmonary fibrosis. Even if patients experience a decline while on these medications, continuing treatment reduces the likelihood of further declines. Side effects, including gastrointestinal issues and skin rashes, are common but manageable. Patients often tolerate mild side effects if the medication is effective. It’s crucial to address lifestyle factors like smoking, weight management, and exercise. Interval training is as effective as continuous training, and supportive care is essential for maintaining quality of life.