Opinion
Video
Current Treatment Paradigms: Efficacy, Safety, and Patient Adherence in IPF and PPF Care
Panelists discuss how effective management of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) relies on early, accurate diagnosis and multidisciplinary collaboration, with a focus on personalized treatment strategies, proactive adverse effect management, and supportive interventions like pulmonary rehabilitation and nutrition to enhance quality of life and improve long-term outcomes.
Current management of IPF and PPF begins with a thorough diagnostic evaluation, including imaging, pulmonary function testing, and sleep studies to identify coexisting conditions that may worsen disease progression or affect oxygen needs. Accurate diagnosis is critical, and multidisciplinary collaboration plays a central role in reducing misdiagnosis and expediting treatment initiation. Follow-up typically occurs every 3 to 6 months, with reassessments triggered by new symptoms or hospitalizations. Early intervention, including pulmonary rehabilitation and oxygen therapy, is emphasized to support functional status and improve quality of life.
Treatment selection must consider both efficacy and tolerability. While current antifibrotic therapies can slow disease progression, they are often associated with gastrointestinal adverse effects, especially in the setting of other comorbidities or polypharmacy. Simplifying medication regimens and proactively managing adverse effects—such as through dietary modifications, dose adjustments, or the use of supportive medications—can enhance adherence. Patient education is essential, particularly around recognizing adverse effects, maintaining consistent medication use, and understanding the importance of lifestyle adjustments, including exercise and nutrition.
Physical activity and pulmonary rehabilitation are increasingly recognized not only for improving exercise tolerance and quality of life but also for potentially extending survival. Addressing malnutrition, muscle wasting, and immune resilience through tailored nutrition plans is also key, especially as patients become more sedentary or experience disease-related weight loss. Overall, proactive and personalized care—rooted in early diagnosis, collaborative treatment planning, and patient empowerment—can significantly affect outcomes in IPF and PPF.
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