Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease

Ryu, Jay H.; Myers, Jeffrey L.; Capizzi, Stephen A.; Douglas, W. W.; Vassallo, Robert; Decker, Paul A.

doi:10.1378/chest.127.1.178

Cited by 189 publications

(192 citation statements)

References 24 publications

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“…The authors of this study did not support the common reduction in DLco in patients with RB-ILD. Normal initial DLco values were demonstrated in 36% of patients, suggesting lower sensitivity of that marker of clinically significant disease than had been previously indicated [6][7][8]12].…”

Section: Physiologic Changesmentioning

confidence: 74%

“…Definitive reports of natural history of RB-ILD are not available due to the relatively small number of patients studied to date [6][7][8]12,13]. A few earlier case series showed that most patients with RB-ILD had a relatively stable clinical course [12].…”

Section: Natural History and Prognosismentioning

confidence: 99%

“…Most insight into RB-ILD to date has been provided by five case series [6][7][8]12,13] totaling 78 patients with biopsy-proven RB-ILD, These studies have provided descriptions of clinical and physiological features, pathological and radiographic/HRCT appearances, and outcomes of the disease. Additionally, there have been several other reports focused mainly on the differential diagnosis of RB-ILD based on radiological findings [9] and their correlation with clinical and physiological findings [14], or on follow-up after smoking cessation [14,15].…”

mentioning

confidence: 99%

“…In spite of the substantial clinical heterogeneity of these studies, it has been consistently reported that patients with RB-ILD are usually ever cigarette smokers with at least 30 pack-years at the time of diagnosis [6][7][8][9]12,13]. Only incidental single cases of RB-ILD in non-smokers have been reported, including patients exposed to second-hand smoke [8,16].…”

mentioning

confidence: 99%

“…Only incidental single cases of RB-ILD in non-smokers have been reported, including patients exposed to second-hand smoke [8,16]. The age at symptom onset ranges from 22 to 70 years, with median values of 36-54 years [6][7][8]12,13]. When the disease occurred at a younger age, it was usually in heavy smokers who had smoked 2 to 3 packs of cigarettes for at least 10 years.…”

mentioning

confidence: 99%

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Respiratory Bronchiolitis with Interstitial Lung Disease

Galanski¹

2010

Thoracic Imaging

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Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is a rare, mild inflammatory pulmonary disorder that occurs almost exclusively in current or former heavy smokers, usually between the third and sixth decades, most likely with no gender predilection. The onset is usually insidious with exertional dyspnea and persistent cough, which may be non-productive, developing over a course of weeks or months. RB-ILD may also be diagnosed in asymptomatic patients with functional impairment and chest radiograph or high-resolution computed tomography (HRCT) abnormalities. Histologically, RB-ILD is characterized by the accumulation of yellow-brown pigmented macrophages within the lumens of respiratory bronchioles and alveolar ducts, associated with a patchy submucosal and peribronchiolar chronic inflammation. Common findings also include mild bronchiolar and peribronchiolar alveolar fibrosis that expands contiguous alveolar septa and leads to architectural distortion as well as centrilobular emphysema. Chest radiographs in patients with RB-ILD typically show fine reticulonodular interstitial opacities, while on HRCT central and peripheral bronchial wall thickening, centrilobular nodules, and ground-glass opacities associated with upper lobe centrilobular emphysema are most frequently reported. Pulmonary function testing may be normal but usually demonstrates mixed, predominantly obstructive abnormalities, often combined with hyperinflation and usually associated with a mild to moderate reduction in carbon monoxide diffusion capacity (DLco). The course of RB-ILD is heterogeneous. Some patients respond favorably to corticosteroids and/or smoking cessation, but often there is no functional improvement and the disease progresses despite smoking cessation and treatment.

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Section: Physiologic Changesmentioning

confidence: 74%