Cardiac multi-detector row CT provides extensive information regarding the size and morphology of the CTI and its related structures.
Original Clinical Science-General Background. Chronic lung allograft dysfunction (CLAD) phenotype determines prognosis and may have therapeutic implications. Despite the clarity achieved by recent consensus statement definitions, their reliance on radiologic interpretation introduces subjectivity. The Center for Computer Vision and Imaging Biomarkers at the University of California, Los Angeles (UCLA) has established protocols for chest high-resolution computed tomography (HRCT)-based computer-aided quantification of both interstitial disease and air-trapping. We applied quantitative image analysis (QIA) at CLAD onset to demonstrate radiographic phenotypes with clinical implications. Methods. We studied 47 first bilateral lung transplant recipients at UCLA with chest HRCT performed within 90 d of CLAD onset and 47 no-CLAD control HRCTs. QIA determined the proportion of lung volume affected by interstitial disease and air-trapping in total lung capacity and residual volume images, respectively. We compared QIA scores between no-CLAD and CLAD, and between phenotypes. We also assigned radiographic phenotypes based solely on QIA, and compared their survival outcomes. Results. CLAD onset HRCTs had more lung affected by the interstitial disease (P = 0.003) than no-CLAD controls. Bronchiolitis obliterans syndrome (BOS) cases had lower scores for interstitial disease as compared with probable restrictive allograft syndrome (RAS) (P < 0.0001) and mixed CLAD (P = 0.02) phenotypes. BOS cases had more air-trapping than probable RAS (P < 0.0001). Among phenotypes assigned by QIA, the relative risk of death was greatest for mixed (relative risk [RR] 11.81), followed by RAS (RR 6.27) and BOS (RR 3.15). Conclusions. Chest HRCT QIA at CLAD onset appears promising as a method for precise determination of CLAD phenotypes with survival implications.
Objective: To investigate the prevalence of the MUC5B promoter variant rs35705950 in patients with systemic sclerosis-interstitial lung disease (SSc-ILD) and whether its presence predicts response to immunosuppression with cyclophosphamide (CYC) and mycophenolate (MMF).Methods: SSc-ILD patients who participated in Scleroderma Lung Study (SLS) II (MMF versus CYC) were included in this study (N=142). TaqMan Genotyping Assays were used to determine the MUC5B rs35705950 single nucleotide polymorphism. Joint models were created to examine how the presence of this variant affected the course of the forced vital capacity (FVC) over 2 years. Linear regression models were used to investigate the relationship between the presence of this variant and the change in quantitative radiographic fibrosis.
Objectives To investigate computer-aided quantitative scores from high-resolution CT (HRCT) images and determine their longitudinal changes and clinical significance in patients with idiopathic inflammatory myopathies (IIMs)-related interstitial lung disease (IIMs-ILD). Methods The clinical data and HRCT images of 80 patients with IIMs who underwent serial HRCT scans at least twice were retrospectively analysed. Quantitative ILD (QILD) scores (%) were calculated as the sum of the extent of lung fibrosis, ground-glass opacity, and honeycombing. The individual time-estimated ΔQILD between two consecutive scans was derived using a linear approximation of yearly changes. Results The baseline median QILD (interquartile range) scores in the whole lung were 28.1% (19.1–43.8). The QILD was significantly correlated with forced vital capacity (r=-0.349, P = 0.002) and diffusing capacity for carbon monoxide (r=-0.381, P = 0.001). For ΔQILD between the first two scans, according to the visual ILD subtype, QILD aggravation was more frequent in patients with usual interstitial pneumonia (UIP) than non-UIP (80.0% vs. 44.4%, P = 0.013). Multivariable logistic regression analyses identified UIP was significantly related to radiographic ILD progression (ΔQILD >2%, P = 0.015). Patients with higher baseline QILD scores (>28.1%) had a higher risk of lung transplantation or death (P = 0.015). In the analysis of three serial HRCT scans (n = 41), dynamic ΔQILD with four distinct patterns (improving, worsening, convex, and concave) was observed. Conclusion QILD changes in IIMs-ILD were dynamic, and baseline UIP patterns seemed to be related to a longitudinal progression in QILD. These may be a potential imaging biomarkers for lung function, changes in ILD severity, and prognosis in IIMs-ILD.
Objective: To explore the presence of small airway disease (SAD) and emphysema in scleroderma-related interstitial lung disease (SSc-ILD) and to evaluate the physiologic and clinical correlates of SAD in SSc-ILD. Methods: Thoracic high-resolution computed tomography (HRCT) images obtained from the Scleroderma Lung Study II (SLSII) participants were reviewed by a group of thoracic radiologists. The presence of SAD was assessed by visual assessment for air trapping. HRCT scans were also evaluated for the presence of emphysema. The association of the presence of air trapping and emphysema with physiological measures of airway disease and clinical variables was evaluated. Results: A total of 155 baseline HRCT scans were reviewed. For assessment of air trapping, images needed to be adequate end-expiratory examinations, leaving 123 scans. Air trapping was seen in 13/123 (10.6%) of the SSc-ILD cohort and was independent of smoking history, asthma or the presence of gastroesophageal reflux. Air trapping on HRCT was not associated with physiologic evidence of SAD. We also identified 8/155 (5.2%) patients with emphysema on HRCT, which was independent of SAD and found mostly in prior smokers. Conclusion: We report the first study of air trapping on standardized, high-quality HRCT images as a reflection of SAD in a relatively large, well characterized SSc-ILD cohort. The presence of SAD in non-smoking SSc-ILD patients supports that SSc may cause not only restrictive lung disease (SSc-ILD), but also, to a lesser extent, obstructive disease. Physiologic measures alone may be inadequate to detect airway disease in patients with SSc-ILD.
BackgroundThe idiopathic inflammatory myopathies (IIM) are autoimmune connective tissue diseases affecting skeletal muscle, skin and other organ systems. IIM-related interstitial lung disease (IIM-ILD) is the most common extra-muscular manifestation, being the leading cause of morbidity and mortality. Several studies have suggested that ILD pattern based on chest high-resolution computed tomography (HRCT) can be related to disease course and treatment response, but the results vary considerably. Moreover, the clinical impact of the quantitative ILD (QILD) score, a validated computer-aided scoring system in assessing ILD severity from HRCT, and its longitudinal changes have not yet been evaluated in IIM-ILD.ObjectivesThis study aims to investigate ILD patterns and QILD scores in patients with IIM-ILD, to identify their clinical impact, and to delineate longitudinal changes of QILD measurement.MethodsA total of 80 patients with IIM (polymyositis 22, and dermatomyositis 58) who underwent at least 2 times of serial HRCT scans were included. Visual ILD patterns were assessed by multiple thoracic radiologists. Quantitative analysis of HRCT was presented as total extent of QILD scores (%) in whole lung and most severe zone. Individual time-estimated ΔQILD score between first 2 visits was derived using a linear approximation of yearly change, where the duration of median (IQR) was 1.0 (0.4-1.6) years in the first 2 HRCT scans.ResultsThe median (IQR) age of the patients was 52.0 (43.5-58.5) years and 60 (75.0%) were women. Baseline median score of whole lung-QILD and most severe zone-QILD were 28.1% (19.1-43.8) and 68.0% (45.5-81.8), respectively, and QILD score showed significant correlations with pulmonary function tests (r=-0.349, p=0.002 for % predicted forced vital capacity; and r=-0.381, p=0.001 for % predicted diffusing capacity for carbon monoxide). The individual time-estimated yearly ΔQILD score between first 2 visits presented that approximately half of the patients showed improvement or stability in QILD scores; however, when patients were sorted by visual assessment in ILD subtype on HRCT, approximately two-thirds of the patients with usual interstitial pneumonia (UIP) pattern were aggravated in QILD scores and less than half of subjects with nonspecific interstitial pneumonia and organizing pneumonia were aggravated (Figure 1, 80% for UIP vs. 44.4% for non-UIP, p=0.013). There was no immunosuppressive drugs related to meaningful improvement in QILD scores during first 2 visits. Notably, we observed significant aggravation of QILD scores in tacrolimus users (n=7, median time-estimated whole lung-yarly ΔQILD 20.3 (2.7-38.4)) compared with tacrolimus non-users (n=73, median time estimated whole lung-yearly ΔQILD -1.2 (-8.3-6.5)). Among 80 patients, 6 (7.5%) were died due to various lung complications. Higher baseline QILD scores were noted in deaths (median whole lung-QILD 45.4 (32.9-56.5)) than in survivors (median whole lung-QILD 26.9 (19.0-42.4)), albeit not significant (p=0.084). Poor survival rate was observed in patients with high grade of ground glass opacity by visual assessment in right upper lobe (log-rank test, p=0.042). Among subgroup of patients with 3 serial HRCT scans (n=41), dynamic changes of four distinct patterns (improving, worsening, convex, and concave) were observed.Figure 1.Cleveland dot plot of individual time-estimated yearly ΔQILD during fist 2 visits.ConclusionThe changes in QILD score in IIM-ILD are dynamic and present different by visual assessment. QILD score has the potential for evaluation of the severity changes, prognosis and medication response in patients with IIM-ILD.References[1]Tashkin DP, et al. Ann Rheum Dis 2016;75(2):374-81.7 truncated values in the graph A. NSIP: nonspecific interstitial pneumonia; OP: organizing pneumonia; UIP: usual interstitial pneumonia.Disclosure of InterestsNone declared
Birt-Hogg-Dubé Syndrome (BHDS) is a rare autosomal dominant disease which manifests with cutaneous hamartomas, lung cysts and renal carcinomas. A wide spectrum of phenotypic expression and few visible manifestations makes BHDS a likely under-recognized entity. Diffuse cystic lung disease (DCLD) is the typical pulmonary manifestation of BHDS, which in the absence of other specific findings carries a broad differential diagnosis. Unlike many other causes of DCLD, BHDS is not known to present with symptomatic pulmonary dysfunction. We report a typical case of BHDS with an atypical presentation – chronic progressive dyspnea. The unusual presentation provides an opportunity to discuss the differential for DCLD and highlights the importance of maintaining an index of suspicion for BHDS even when symptoms appear inconsistent with the diagnosis. Also examined is the management of BHDS patients and their immediate relatives, and recommendations for the treatment of obstructive sleep apnea (OSA) with continuous positive airway pressure (CPAP) given the potential risk of pneumothorax in this group.
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