Book of PDFs-ILD Main Case Part II

ILD Main Case Part II

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This document discusses the approach to interstitial lung diseases (ILD) using a multidisciplinary approach. The case presented is of a 60-year-old woman who presents with cough and dyspnea for 4 months. The first step in evaluating her ILD further would be a detailed history and physical examination. The CT pattern diagnosis is probable UIP according to the 2011 ATS-ERS criteria. The likelihood of definite or probable UIP on histopathology in this case is 80%. There are problems with the current classification of possible UIP, and a reclassification into probable UIP and indeterminate UIP is being considered. To confirm the patient's diagnosis, a surgical lung biopsy was performed which showed usual interstitial pneumonia (UIP) pattern. The final clinical diagnosis based on the multidisciplinary input is idiopathic pulmonary fibrosis (IPF). Treatment options for IPF include anti-fibrotic therapy with either pirfenidone or nintedanib. Anti-fibrotic therapy can be offered even in the absence of symptoms with stable PFTs. If symptoms worsen or there is a significant drop in lung function, switching to an alternative anti-fibrotic therapy can be considered. In the case of an IPF exacerbation, treatment with methylprednisolone followed by prednisone and supportive care is recommended.

Keywords

Interstitial lung diseases

Multidisciplinary approach

Probable UIP

Histopathology

Surgical lung biopsy

Idiopathic pulmonary fibrosis

Anti-fibrotic therapy

Pirfenidone

Nintedanib

IPF exacerbation