PG 2025: Pulmonary Literature Review and Interactive Complex Case Presentations (PG)-Chan KM

Chan KM - Interactive Complex Case Presentations

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This case presentation by Dr. Kevin M. Chan discusses an 84-year-old woman with interstitial lung disease (ILD) complicated by bronchiectasis, presenting initially with cough and dyspnea triggered by swimming. Despite treatment with inhaled corticosteroids and a pulmonary clearance device, symptoms did not improve. Initial examination revealed stable oxygen saturation and clear lungs. Pulmonary function tests and high-resolution CT scans were utilized in evaluation. <br /><br />Nine months later, the patient experienced worsening symptoms including night sweats, cough, and increased dyspnea, now occurring at rest. Physical exam showed hypoxemia (90% on room air), bibasilar crackles, and fatigue. Broad infectious workup, bronchoscopy with bronchoalveolar lavage, and cultures were negative. Differential diagnoses considered included community-acquired pneumonia, organizing pneumonia, acute exacerbation of ILD, eosinophilic pneumonia, and helminthic infection. Treatment included antibiotics, corticosteroids, and Pneumocystis jirovecii pneumonia (PJP) prophylaxis. She was discharged on supplemental oxygen.<br /><br />The diagnosis was acute exacerbation of nonspecific interstitial pneumonia (NSIP). Literature review reveals that corticosteroid therapy in acute exacerbations of ILD (AE-ILD) shows potential survival benefit in non-IPF ILD, but inconsistent or harmful effects in idiopathic pulmonary fibrosis (IPF). A 2025 systematic review indicated high-dose steroids improve survival and reduce 90-day mortality in non-IPF ILD, while a meta-analysis reported an increased mortality risk (78% higher) with steroids in AE-IPF. Therefore, corticosteroid use requires individualized risk-benefit assessment.<br /><br />Risk factors for acute exacerbations in progressive pulmonary fibrosis (PPF) resemble those in IPF, including age >65, lower DLCO%, and lack of antifibrotic therapy (e.g., nintedanib). Median survival post-exacerbation is about 300 days.<br /><br />In summary, acute exacerbations in ILD pose significant management challenges. Current evidence supports tailored corticosteroid use in non-IPF patients while exercising caution in IPF. Ongoing monitoring and antifibrotic therapies remain important aspects of care.

Keywords

interstitial lung disease

bronchiectasis

acute exacerbation

nonspecific interstitial pneumonia

corticosteroid therapy

idiopathic pulmonary fibrosis

pulmonary function tests

high-resolution CT

Pneumocystis jirovecii pneumonia prophylaxis

antifibrotic therapy