Book of PDFs-ILD Main Case

ILD Main Case

Pdf Summary

This document discusses a multidisciplinary approach to evaluating and diagnosing interstitial lung diseases (ILDs). It presents a case study of a 60-year-old woman with a progressive cough and dyspnea on exertion. The first step in evaluating her ILD is to perform a detailed history and physical examination. The CT pattern diagnosis according to ATS-ERS criteria is "probable UIP." The likelihood of definite or probable UIP on histopathology is 80%. Current classification of possible UIP is being reevaluated to improve accuracy. To confirm the diagnosis, a surgical lung biopsy is performed, revealing usual interstitial pneumonia (UIP) pattern. The final clinical diagnosis is "probable idiopathic pulmonary fibrosis" based on multidisciplinary input. Treatment options for IPF include prednisone, azathioprine, N-acetyl cysteine, pirfenidone, and nintedanib. Anti-fibrotic therapy, such as pirfenidone or nintedanib, is recommended for patients with confirmed IPF and progressive symptoms. Stability of symptoms and PFTs indicates that the medication is working. If symptoms worsen or there is a decline in FVC, switching to an alternative anti-fibrotic therapy may be considered. In the case of an IPF exacerbation, treatment with methylprednisolone or prednisone is recommended. Supportive/palliative care is an option for those who do not respond to treatment or have poor prognosis.

Keywords

interstitial lung diseases

ILDs

probable UIP

usual interstitial pneumonia

probable idiopathic pulmonary fibrosis

treatment options

anti-fibrotic therapy

progressive symptoms

alternative anti-fibrotic therapy

supportive/palliative care