Bonus Material-07_Ryu_Diffuse Parenchymal Lung Diseases 2025

Pdf Summary

Diffuse Parenchymal Lung Diseases (DPLDs), also called interstitial lung diseases (ILDs), encompass a heterogeneous group of lung conditions affecting the parenchyma, with a prevalence of about 200 per 100,000 in high-income countries. They include idiopathic interstitial pneumonias (IIPs) like idiopathic pulmonary fibrosis (IPF), ILDs linked to systemic autoimmune rheumatic diseases (SARDs/CTDs), granulomatous ILDs such as sarcoidosis, smoking-related ILDs, and rarer diseases. Diagnosis involves clinical evaluation, pulmonary function testing, high-resolution CT (HRCT), bronchoalveolar lavage (BAL), and sometimes lung biopsy with multidisciplinary discussion.<br /><br />IPF is a progressive fibrosing ILD of unknown cause, primarily diagnosed by typical HRCT findings of usual interstitial pneumonia (UIP) pattern and excluding other causes. It typically affects older adults with symptoms of chronic dyspnea and cough, basal crackles on auscultation, and has a median survival around three years. Genetic factors such as telomere syndromes, smoking, environmental exposures, and aberrant epithelial repair contribute to pathogenesis. Treatment includes antifibrotic therapies (pirfenidone, nintedanib) that slow lung function decline, supportive care, managing comorbidities (e.g., GERD, pulmonary hypertension), and lung transplantation.<br /><br />Other IIPs include nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), respiratory bronchiolitis-associated ILD, and rarer types. Smoking-related ILDs (e.g., PLCH, DIP) are closely linked to tobacco exposure. SARD-associated ILDs occur frequently, with patterns dependent on the underlying autoimmune condition (e.g., RA, systemic sclerosis). Management involves immunosuppression, antifibrotic drugs, and addressing disease-specific manifestations.<br /><br />Sarcoidosis is a multisystem granulomatous disease of unknown cause featuring noncaseating granulomas primarily affecting lungs and lymph nodes, with variable prognosis. Diagnosis is by compatible clinical-radiologic features and exclusion of other granulomatous diseases.<br /><br />Emerging concepts include interstitial lung abnormalities (ILA) that may precede overt ILD and progressive pulmonary fibrosis (PPF) in non-IPF fibrotic ILDs, warranting consideration of antifibrotic therapy.<br /><br />In sum, DPLDs require a comprehensive approach to diagnosis, understanding diverse etiologies, and tailored treatments to improve patient outcomes.

Meta Tag

Concept Diffuse Parenchymal Lung Disease

Concept Interstitial Lung Disease

Concept Idiopathic Interstitial Pneumonia

Concept Idiopathic Pulmonary Fibrosis

Concept Systemic Autoimmune Rheumatic Disease

Keywords

Diffuse parenchymal lung diseases

Interstitial lung diseases

Idiopathic pulmonary fibrosis

High-resolution CT

Usual interstitial pneumonia

Antifibrotic therapy

Sarcoidosis

Systemic autoimmune rheumatic diseases

Progressive pulmonary fibrosis

Bronchoalveolar lavage

Diffuse Parenchymal Lung Disease

Interstitial Lung Disease

Idiopathic Interstitial Pneumonia

Idiopathic Pulmonary Fibrosis

Systemic Autoimmune Rheumatic Disease