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Idiopathic Pulmonary Fibrosis: A Comprehensive Overview of Diagnosis and Treatment

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Prof. Dr. BN BM Prasad, Senior Consultant Pulmonary Medicine, Interventional Pulmonology, Sleep Medicine, Chinmaya Mission Hospital, Bangalore    27 February 2025

Idiopathic pulmonary fibrosis (IPF) is a progressive and debilitating lung disease that carries a high mortality rate. Primarily affecting middle-aged and elderly individuals, the condition is more prevalent among males. Various factors, including occupational exposures, connective tissue disorders, and certain medications, contribute to the complexity of diagnosing IPF. It falls under the broader category of interstitial lung diseases (ILDs), which encompass various lung and heart complications.

The understanding of IPF has evolved significantly from the 17th century to contemporary times, with advancements in diagnostic classifications playing a crucial role in managing the disease. Environmental factors, occupational history, and genetic predispositions are key in diagnosing ILDs and distinguishing IPF from other similar diseases. Common causes of ILDs include connective tissue disorders, smoking-related lung fibrosis, and environmental exposures, which can lead to conditions like hypersensitivity pneumonitis. Differentiating IPF from other forms of interstitial pneumonias, such as non-specific interstitial pneumonia and usual interstitial pneumonia, requires tailored management strategies.

Accurate diagnosis of IPF depends on a detailed patient history, including occupational and family background, and advanced imaging techniques like CT scans. These imaging methods have revolutionized the ability to detect and classify interstitial abnormalities based on their distribution, aiding in the identification of distinct pathologies and guiding treatment decisions. Key diagnostic indicators of IPF include the presence of usual interstitial pneumonia (UIP) patterns on high-resolution CT scans, and in some cases, lung biopsy specimens. A surgical biopsy may not be necessary if classic IPF features are evident through imaging.

The management of IPF revolves around antifibrotic drugs, such as Nintedanib, which inhibits fibroblastic activity and new blood vessel formation by targeting tyrosine kinase and vascular endothelial growth factors. While Nintedanib does not cure fibrosis, it plays a crucial role in stabilizing lung function. Administered as soft capsules in 100-150 mg doses taken twice daily with food, the drug’s side effects can include nausea, vomiting, diarrhea, weight loss, skin rashes, and respiratory tract infections. These side effects may require dose adjustments and supportive treatments. Furthermore, Nintedanib’s efficacy can be reduced by tobacco use, and its bioavailability can be affected by drugs that influence liver enzymes, warranting careful management of drug interactions.

Early and accurate diagnosis, along with appropriate management of IPF, including lifestyle modifications and monitoring drug interactions, is essential for improving patient outcomes.

Key Takeaways

  • Idiopathic pulmonary fibrosis (IPF) is a severe and progressive lung disease with a high mortality rate, requiring early and accurate diagnosis.
  • Understanding the pathogenesis and classification of IPF is crucial for effective management and treatment.
  • Advanced imaging techniques, such as CT scans, are pivotal in diagnosing and subtyping IPF, guiding treatment decisions.
  • Antifibrotic drugs, like Nintedanib, stabilize lung function but do not cure fibrosis, requiring careful management of side effects and drug interactions.
  • A detailed patient history and consideration of environmental and genetic factors are essential for differentiating IPF from other interstitial lung diseases.

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