Panelists discuss how progressive pulmonary fibrosis (PPF) and idiopathic pulmonary fibrosis (IPF), though distinct in etiology, share a common trajectory of irreversible lung scarring and functional decline—highlighting the importance of recognizing progressive phenotypes across interstitial lung diseases to guide timely diagnosis, personalized treatment, and improved patient outcomes.
An interdisciplinary approach to diagnosing IPF and PPF plays a pivotal role in improving both accuracy and timeliness. Early and correct diagnosis is critical, given the short median survival—often only 3 to 5 years—for these patients. Many individuals present with nonspecific symptoms like cough and exertional shortness of breath, which can be mistaken for other common conditions. Delays in diagnosis, sometimes extending over 2 years from initial symptoms to confirmation, severely limit treatment options and decrease survival chances. Multidisciplinary collaboration ensures that patients are evaluated from multiple angles, expediting diagnosis and treatment.
Effective interdisciplinary care involves pulmonologists, radiologists, pathologists, and often rheumatologists working in close coordination. Rather than treating patients in isolated specialties, a collective team reviews imaging, clinical presentation, and underlying conditions such as autoimmune diseases. This ensures differential diagnoses are thoroughly considered and that the right diagnostic pathways are followed. In diseases like systemic sclerosis or rheumatoid arthritis, where pulmonary complications may be silent early on, regular screening and communication between providers can lead to earlier detection of interstitial lung disease. This proactive approach is particularly important because once fibrosis advances, lung damage is irreversible.
Beyond diagnosis, interdisciplinary care also enhances management. Rheumatologists monitoring systemic diseases can refer patients promptly when signs of lung involvement arise. Radiologists help interpret subtle changes on high-resolution CT scans, and pulmonologists guide treatment selection, including the use of antifibrotic therapies. This model not only improves clinical outcomes but also reduces unnecessary hospitalizations and delays. Overall, the collaboration across specialties helps optimize care delivery, ensuring that patients with IPF or PPF receive timely, targeted interventions that may extend life and improve quality of care.