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The 2025 ERS/ATS statement states that patients with predominant bronchiolitis features in the macrophage-accumulation spectrum are referred to as RB-ILD. It reports that two large cohort studies found 93% of histological RB-ILD-pattern cases to be smoking-related, with only a small minority considered idiopathic. It emphasizes that RB is common and incidental in smokers when not accompanied by symptoms or abnormal physiology. It also states that BAL with lightly pigmented macrophages can support RB-ILD in the correct clinical and imaging context and may help exclude non-fibrotic hypersensitivity pneumonitis. Details
The 2025 ERS/ATS statement classifies RB-ILD under alveolar filling disorders and introduces updated terminology, replacing desquamative interstitial pneumonia (DIP) with alveolar macrophage pneumonia (AMP) and defining diagnostic confidence levels (confident, provisional, unclassifiable). Details
This review defines RB-ILD as a rare inflammatory pulmonary disorder occurring almost exclusively in current or former heavy smokers, usually between the third and sixth decades. It summarizes typical manifestations as dyspnea, cough, obstructive/restrictive/mixed pulmonary function abnormalities, and HRCT centrilobular micronodules, ground-glass opacities and peribronchiolar thickening with smoking-related emphysema. It reports that the main evidence base consists of five case series totaling 78 biopsy-proven patients. It also summarizes Portnoy physiology data, including obstructive defects in 47%, restriction in 31%, mixed defects in 9%, normal spirometry in 13%, and normal initial DLCO in 36%. Details
The 2024 review describes RB-ILD as an anatomopathologic smoking-related respiratory bronchiolitis lesion that becomes clinically relevant when symptoms, HRCT abnormalities and functional impairment appear. It reports RB-ILD risk as smoking in >95% and typical age 30–60 years, with HRCT centrilobular nodules, bronchial wall thickening and bronchiolocentric distribution. It states that lung function may be normal or show low DLCO, and BAL is nonspecific but may show smoker’s macrophages. The review considers smoking cessation the main treatment, reserving corticosteroids for progression despite cessation, but this is based on low-level evidence. Details
The Korean guideline section on other ILDs addresses RB-ILD and DIP as smoking-related disorders and notes that recommendations are consensus-based because randomized trials are insufficient. It explicitly recommends smoking cessation as a first-line strategy for RB-ILD. It describes RB as a smoking-related histologic finding observed in up to 89% of smokers with lung biopsy for reasons unrelated to RB. The guideline also notes that RB-ILD generally has long-term survival, although treatment response and clinical course vary. Details
The 2025 ERS/ATS statement states that patients with predominant bronchiolitis features in the macrophage-accumulation spectrum are referred to as RB-ILD. It reports that two large cohort studies found 93% of histological RB-ILD-pattern cases to be smoking-related, with only a small minority considered idiopathic. It emphasizes that RB is common and incidental in smokers when not accompanied by symptoms or abnormal physiology. It also states that BAL with lightly pigmented macrophages can support RB-ILD in the correct clinical and imaging context and may help exclude non-fibrotic hypersensitivity pneumonitis. Details
The 2025 ERS/ATS statement classifies RB-ILD under alveolar filling disorders and introduces updated terminology, replacing desquamative interstitial pneumonia (DIP) with alveolar macrophage pneumonia (AMP) and defining diagnostic confidence levels (confident, provisional, unclassifiable). Details
This review defines RB-ILD as a rare inflammatory pulmonary disorder occurring almost exclusively in current or former heavy smokers, usually between the third and sixth decades. It summarizes typical manifestations as dyspnea, cough, obstructive/restrictive/mixed pulmonary function abnormalities, and HRCT centrilobular micronodules, ground-glass opacities and peribronchiolar thickening with smoking-related emphysema. It reports that the main evidence base consists of five case series totaling 78 biopsy-proven patients. It also summarizes Portnoy physiology data, including obstructive defects in 47%, restriction in 31%, mixed defects in 9%, normal spirometry in 13%, and normal initial DLCO in 36%. Details
The 2024 review describes RB-ILD as an anatomopathologic smoking-related respiratory bronchiolitis lesion that becomes clinically relevant when symptoms, HRCT abnormalities and functional impairment appear. It reports RB-ILD risk as smoking in >95% and typical age 30–60 years, with HRCT centrilobular nodules, bronchial wall thickening and bronchiolocentric distribution. It states that lung function may be normal or show low DLCO, and BAL is nonspecific but may show smoker’s macrophages. The review considers smoking cessation the main treatment, reserving corticosteroids for progression despite cessation, but this is based on low-level evidence. Details
The Korean guideline section on other ILDs addresses RB-ILD and DIP as smoking-related disorders and notes that recommendations are consensus-based because randomized trials are insufficient. It explicitly recommends smoking cessation as a first-line strategy for RB-ILD. It describes RB as a smoking-related histologic finding observed in up to 89% of smokers with lung biopsy for reasons unrelated to RB. The guideline also notes that RB-ILD generally has long-term survival, although treatment response and clinical course vary. Details
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