Smoking-related interstitial lung disease: radiologic-clinical-pathologic correlation

Abstract

Cigarette smoking is a recognized risk factor for development of interstitial lung disease (ILD). There is strong evidence supporting a causal role for cigarette smoking in development of respiratory bronchiolitis ILD (RB-ILD), desquamative interstitial pneumonitis (DIP), and pulmonary Langerhans cell histiocytosis (PLCH). In addition, former and current smokers may be at increased risk for developing idiopathic pulmonary fibrosis (IPF). The combination of lower lung fibrosis and upper lung emphysema is being increasingly recognized as a distinct clinical entity in smokers. High-resolution computed tomography is sensitive for detection and characterization of ILD and may allow recognition and classification of the smoking-related ILDs (SR-ILDs) into distinct individual entities. However, the clinical, radiologic, and histologic features overlap among the different SR-ILDs, and mixed patterns of disease frequently coexist in the same patient. The overlap is most significant between RB-ILD and DIP. Macrophage accumulation is bronchiolocentric in RB-ILD, producing centrilobular ground-glass opacity, and more diffuse in DIP, producing widespread ground-glass changes. The coexistence of upper lung nodules and cysts in a smoker allows confident diagnosis of PLCH. Final diagnosis of an SR-ILD and identification of the specific entity can be achieved with certainty only after the pulmonologist, radiologist, and pathologist have reviewed all of the clinical, radiologic, and pathologic data.