Diagnostic accuracy of pelvis multiparametric MRI against CT virtual hysterosalpingography: a prospective study of tubal patency through female infertility assessment.

OBJECTIVE: To evaluate the diagnostic accuracy of MRI-hysterosalpingogram (HSG) with semiquantitative dynamic contrast-enhanced perfusion, against the virtual multislice CT hysterosalpingogram (VHSG) as a reference standard. METHODS AND MATERIALS: In this prospective study, 26 women (age >18 years) searching for infertility causes and with VHSG physician request. Thereafter, the assessment performance of both techniques was determined by two reader analyses. k statistics were used for the assessment of tubal patency. Receiver operating characteristic (ROC) analysis was used to compare the capability for tubal patency assessment between both exams on a per-patient and per-tube basis. The McNemar test was used to compare the diagnostic accuracy measures. RESULTS: Tubal patency, uterine morphological, ovarian, and extrauterine abnormalities were evaluated through both exams in all 26 women. There was no significant difference between diagnostic performance measurements between the methods. The ROC curve of VHSG was 0.852 for both per-patient and per-tube analyses, and one and 0.938 for MRI-HSG. Sensitivity and specificity for per-patient and per-tube for VHSG were 95.2 and 97.7, 80 and 87.5%, and for MRI-HSG 100% for both analyses and 100 and 87.5%, respectively. CONCLUSION: This study demonstrates the feasibility of diagnosing tubal patency through MRI, using a semi-quantitative dynamic contrast-enhanced perfusion sequence, and the satisfactory diagnosing of the uterine morphology, ovarian abnormalities, and ovarian and deep endometriosis. ADVANCES IN KNOWLEDGE: Multiparametric MRI with a perfusion real-time sequence as a HSG method can be used in the evaluation not only for uterine and ovarian abnormilities but also tubal patency.

Postoperative computed tomography of insufflated lung specimens obtained by video-assisted thoracic surgery: detection and margin assessment of pulmonary nodules.

Objective: To investigate the utility of computed tomography (CT) scans to detect and assess the margin status of pulmonary nodules that were insufflated after being resected by video-assisted thoracic surgery. Materials and Methods: This was a novel multicenter study conducted at two national referral centers for thoracic diseases. Patients suspected of having lung cancer underwent video-assisted thoracic surgery for the resection of pulmonary nodules, which were submitted to postoperative CT. Measurements from the CT scans were compared with the results of the histopathological analysis. Results: A total of 37 pulmonary nodules from 37 patients were evaluated. The mean age of the patients was 65 years (range, 36-84 years), and 27 (73%) were female. A CT analysis of insufflated specimens identified all 37 nodules, and 33 of those nodules were found to have tumor-free margins. The histopathological analysis revealed lung cancer in 30 of the nodules, all with tumor-free margins, and benign lesions in the seven remaining nodules. Conclusion: Postoperative CT of insufflated suspicious lung lesions provides real-time detection of pulmonary nodules and satisfactory assessment of tumor margins. This initial study shows that CT of insufflated lung lesions can be a valuable tool at centers where intraoperative histopathological analysis is unavailable.

Objetivo: Investigar a utilidade da tomografia computadorizada (TC) para a detecção e avaliação de margens de nódulos pulmonares que foram insuflados após ressecção por cirurgia torácica videoassistida. Materiais e Métodos: Um inédito estudo multicêntrico foi conduzido em dois centros de referência nacional para doenças torácicas. Nódulos foram ressecados por cirurgia torácica videoassistida de pacientes com suspeita de câncer de pulmão e submetidos a TC pós-operatória. As medidas radiológicas da TC foram comparadas com as da análise patológica. Resultados: Um total de 37 pacientes foi avaliado. A idade média foi de 65 anos (variação: 36-84 anos) e 27 indivíduos (73%) eram do sexo feminino. A análise por TC dos espécimes insuflados identificou todas as 37 lesões e 33 delas com margens livres. A análise patológica revelou 30 casos de câncer de pulmão, todos com margens livres, e sete lesões não malignas. Conclusão: A TC pós-operatória de lesões pulmonares insufladas com suspeita de malignidade provê detecção em tempo real de nódulos pulmonares e aceitável avaliação de margens tumorais. Este estudo inicial demonstra que a TC de lesões pulmonares insufladas pode ser uma ferramenta valiosa em centros em que a análise histopatológica intraoperatória é indisponível.

Postoperative computed tomography of insufflated lung specimens obtained by video-assisted thoracic surgery: detection and margin assessment of pulmonary nodules / Tomografia computadorizada pós-operatória de espécimes pulmonares insuflados após cirurgia torácica videoassistida: detecção e avaliação de margens de nódulos pulmonares

Abstract Objective: To investigate the utility of computed tomography (CT) scans to detect and assess the margin status of pulmonary nodules that were insufflated after being resected by video-assisted thoracic surgery. Materials and Methods: This was a novel multicenter study conducted at two national referral centers for thoracic diseases. Patients suspected of having lung cancer underwent video-assisted thoracic surgery for the resection of pulmonary nodules, which were submitted to postoperative CT. Measurements from the CT scans were compared with the results of the histopathological analysis. Results: A total of 37 pulmonary nodules from 37 patients were evaluated. The mean age of the patients was 65 years (range, 36-84 years), and 27 (73%) were female. A CT analysis of insufflated specimens identified all 37 nodules, and 33 of those nodules were found to have tumor-free margins. The histopathological analysis revealed lung cancer in 30 of the nodules, all with tumor-free margins, and benign lesions in the seven remaining nodules. Conclusion: Postoperative CT of insufflated suspicious lung lesions provides real-time detection of pulmonary nodules and satisfactory assessment of tumor margins. This initial study shows that CT of insufflated lung lesions can be a valuable tool at centers where intraoperative histopathological analysis is unavailable.

Resumo Objetivo: Investigar a utilidade da tomografia computadorizada (TC) para a detecção e avaliação de margens de nódulos pulmonares que foram insuflados após ressecção por cirurgia torácica videoassistida. Materiais e Métodos: Um inédito estudo multicêntrico foi conduzido em dois centros de referência nacional para doenças torácicas. Nódulos foram ressecados por cirurgia torácica videoassistida de pacientes com suspeita de câncer de pulmão e submetidos a TC pós-operatória. As medidas radiológicas da TC foram comparadas com as da análise patológica. Resultados: Um total de 37 pacientes foi avaliado. A idade média foi de 65 anos (variação: 36-84 anos) e 27 indivíduos (73%) eram do sexo feminino. A análise por TC dos espécimes insuflados identificou todas as 37 lesões e 33 delas com margens livres. A análise patológica revelou 30 casos de câncer de pulmão, todos com margens livres, e sete lesões não malignas. Conclusão: A TC pós-operatória de lesões pulmonares insufladas com suspeita de malignidade provê detecção em tempo real de nódulos pulmonares e aceitável avaliação de margens tumorais. Este estudo inicial demonstra que a TC de lesões pulmonares insufladas pode ser uma ferramenta valiosa em centros em que a análise histopatológica intraoperatória é indisponível.

Accuracy of multidetector computed tomography with maximum intensity projection technique in the assessment of fissure integrity.

BACKGROUND: Bronchoscopic lung volume reduction (BLVR) is a potential treatment for patients with severe emphysema, performed through the placement of unidirectional endobronchial valves (EBVs). Their benefits are only achieved in patients that significantly reduce lobar volume, and it is mandatory that the fissures are complete. Fissure evaluation is preferably done by computed tomography, but little is known if its evaluation corresponds to the anatomical findings. The aim of this study is to evaluate the accuracy of thoracic radiologists in the identification of complete fissures by multidetector computed tomography (MDCT) using maximum intensity projection (MIP) technique, compared with direct anatomical evaluation. METHODS: Prospective study, conducted in a single institution. Patients submitted to thoracic surgery had their fissures classified as complete or incomplete by thoracic surgeons and their preoperative chest scans evaluated by three radiologists, blinded for surgical evaluation. With the intraoperative categorization as a reference, the accuracy and concordance of the three thoracic radiologists' evaluation were calculated. The most experienced radiologist evaluated the fissures at two different moments to estimate the intra-observer agreement. RESULTS: There were included 67 patients, being 37 (55%) males, with a mean age of 64 years. The accuracy of radiological identification of complete fissures ranged from 76.8% for left posterior oblique fissure to 85.1% for left anterior oblique fissure, with the best performance achieved by the most experienced radiologist. The concordance of the radiological evaluation for fissure integrity compared to the surgical assessment (k) was 0.53-0.68. Intra-observer agreement ranged from 0.74 to 0.87. CONCLUSIONS: The evaluation of the fissure integrity by MDCT analysis using MIP technique by thoracic radiologists had high accuracy among the thoracic radiologists.

Chest MRI with CT in the assessment of interstitial lung disease progression in patients with systemic sclerosis.

OBJECTIVE: To describe the performance of CT and MRI in the assessment of the progression of interstitial lung disease (ILD) associated with SSc and demonstrate the correlations of MRI with pulmonary function test (PFT) and CT scores. METHODS: This prospective single-centre observational study included patients with SSc diagnoses, and magnetic resonance (MR) images were assessed visually using the Scleroderma Lung Study (SLS) I system. Differences in the median scores were assessed with Student's t-test and the Wilcoxon rank-sum test. Pearson's and Spearman's rank correlation coefficients were calculated to correlate imaging scores and PFT results. Using disease progression as the gold standard, we calculated the area under the curve (AUC) of the CT and MRI scores with Harrel's c-index. The best thresholds for the prediction of disease progression were determined by receiver operating characteristic curve analysis with maximum Youden's Index (P < 0.05). The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of the scores were calculated. RESULTS: The AUCs for MRI and CT scores were 0.86 (0.72-0.98; P = 0.04) and 0.83 (0.70-0.99; P = 0.05), respectively. CT and MRI scores correlated with Forced vital capacity (%FVC) (MRI: r = -0.54, P = 0.0045; CT: r = -0.44; P = 0.137) and diffusing capacity of the lung for carbon monoxide (MRI: r = -0.39, P = 0.007; CT r = -0.36, P = 0.006). The sensitivity, specificity, PPV and NPV were 85%, 87.5%, 88.34% and 86.11% (MRI score) and 84.21%, 82.35%, 84.14% and 82.4% (CT score), respectively. CONCLUSIONS: MRI scores from patients with SSc may be an alternative modality for the assessment of ILD progression in patients with SSc.

Utilization of Quantitative Computed Tomography Assessment to Identify Bronchiolitis Obliterans Syndrome After Single Lung Transplantation.

OBJECTIVE: To evaluate quantitative chest computed tomography (CT) methods for the detection of air trapping (AT) and to assess its diagnostic performance for the diagnosis of bronchiolitis obliterans syndrome (BOS) in single lung transplant (SLT) patients. METHODS: Adult patients who had a SLT at a single transplant center and underwent CT scan after transplantation were retrospectively included. CT findings of air trapping were measured by three different methods: expiratory air-trapping index (ATIexp), mean lung density on expiratory acquisition (MLDexp) and expiratory to inspiratory ratio of mean lung density (E/I-ratio(MLD). Sensitivity, specificity and diagnostic accuracy of the three methods for the detection of BOS status evaluated by serial routine measures of pulmonary function tests (gold standard) were assessed. RESULTS: Forty-six SLT patients (52.2% females, mean age 58 ± 6 years) were included in the analysis, 12 (26%) patients with a diagnosis of BOS. Quantitative CT diagnosis of AT ranged from 26 to 35%. Sensitivity, specificity and accuracy of each method for the detection of BOS were 85.7%, 84.7% and 85.0% for ATIexp, 78.5%, 93.4% and 90.0% for MLD and 64.2%, 89.1% and 83.3% E/I-ratio(MLD), respectively. CONCLUSION: Quantitative measures of AT obtained from standard CT are feasible and show high specificity and accuracy for the detection of BOS in SLT patients.

Ultra-low-dose chest computed tomography without anesthesia in the assessment of pediatric pulmonary diseases / Tomografia computadorizada de tórax de ultrabaixa dosagem sem anestesia naavaliação de doenças pulmonares pediátricas

Abstract Objective To evaluate the feasibility of using ultra-low-dose computed tomography of the chest with iterative reconstruction without anesthesia for assessment of pulmonary diseases in children. Methods This prospective study enrolled 86 consecutive pediatric patients (ranging from 1 month to 18 years) that underwent ultra-low-dose computed tomography due to suspicion of pulmonary diseases, without anesthesia and contrast. Parameters used were: 80 kVp; 15-30 mA; acquisition time, 0.5 s; and pitch, 1.375. The adaptive statistical iterative reconstruction technique was used. Subjective visual evaluation and quantitative assessment of image quality were done using a 5-point scale in 12 different structures of the chest. Results Mean age was 66 months (interquartile range, 16-147). Final diagnosis was performed in all exams, and 44 (51.2%) were diagnosed with cystic fibrosis, 27 (31.4%) with bronchiolitis obliterans, and 15 (17.4%) with congenital pulmonary airways malformations. Diagnostic quality was achieved in 98.9%, of which 82.6% were considered excellent and 16.3% were slightly blurred but did not interfere with image evaluation. Only one case (1.2%) presented moderate blurring that slightly compromised the image, and previous examinations demonstrated findings compatible with bronchiolitis obliterans. Mean effective radiation dose was 0.39 ± 0.15 mSv. Percentages of images with motion artifacts were 0.3% for cystic fibrosis, 1.3% for bronchiolitis obliterans, and 1.1% for congenital pulmonary airways malformations. Conclusion Chest ultra-low-dose computed tomography without sedation or anesthesia delivering a sub-millisievert dose can provide image quality to allow identification of common pulmonary anatomy and diseases.

Resumo Objetivo Avaliar a viabilidade do uso de tomografia computadorizada com ultrabaixa dose com reconstrução iterativa sem anestesia para avaliação de doenças pulmonares em crianças. Métodos Este estudo prospectivo envolveu 86 pacientes pediátricos consecutivos (um mês a 18 anos) submetidos à tomografia computadorizada com ultrabaixa dose por suspeita de doenças pulmonares, sem anestesia e contraste. Os parâmetros utilizados foram: 80 kVp; 15-30 mA; tempo de aquisição, 0,5 s; e pitch de 1,375. Foi utilizada a técnica de reconstrução estatística adaptativa iterativa. A avaliação visual subjetiva e a avaliação quantitativa da qualidade da imagem foram feitas com uma escala de 5 pontos em 12 estruturas do tórax. Resultados A média de idade foi de 66 meses (intervalo interquartil, 16-147). O diagnóstico final foi feito em todos os exames e 44 (51,2%) foram diagnosticados com fibrose cística, 27 (31,4%) com bronquiolite obliterante e 15 (17,4%) com malformação congênita pulmonar das vias aéreas. A qualidade diagnóstica foi alcançada em 98,9% dos casos, dos quais 82,6% foram considerados excelentes e 16,3% alteração leve na definição, mas isso não interferiu na avaliação da imagem. Apenas um caso (1,2%) apresentou alteração moderada na definição, comprometeu discretamente a imagem, e exames prévios demonstraram achados compatíveis com bronquiolite obliterante. A dose de radiação média efetiva foi de 0,39 ± 0,15 mSv. As porcentagens de imagens com artefatos de movimento foram de 0,3% para fibrose cística, 1,3% para bronquiolite obliterante e 1,1% para malformação congênita pulmonar das vias aéreas. Conclusão É possível realizar a tomografia computadorizada com ultrabaixa dose torácica sem sedação ou anestesia, administrando uma dose de submilisievert, com qualidade de imagem suficiente para a identificação pulmonar anatômica e de doenças pulmonares comuns.

Ultra-low-dose chest computed tomography without anesthesia in the assessment of pediatric pulmonary diseases.

OBJECTIVE: To evaluate the feasibility of using ultra-low-dose computed tomography of the chest with iterative reconstruction without anesthesia for assessment of pulmonary diseases in children. METHODS: This prospective study enrolled 86 consecutive pediatric patients (ranging from 1 month to 18 years) that underwent ultra-low-dose computed tomography due to suspicion of pulmonary diseases, without anesthesia and contrast. Parameters used were: 80kVp; 15-30mA; acquisition time, 0.5s; and pitch, 1.375. The adaptive statistical iterative reconstruction technique was used. Subjective visual evaluation and quantitative assessment of image quality were done using a 5-point scale in 12 different structures of the chest. RESULTS: Mean age was 66 months (interquartile range, 16-147). Final diagnosis was performed in all exams, and 44 (51.2%) were diagnosed with cystic fibrosis, 27 (31.4%) with bronchiolitis obliterans, and 15 (17.4%) with congenital pulmonary airways malformations. Diagnostic quality was achieved in 98.9%, of which 82.6% were considered excellent and 16.3% were slightly blurred but did not interfere with image evaluation. Only one case (1.2%) presented moderate blurring that slightly compromised the image, and previous examinations demonstrated findings compatible with bronchiolitis obliterans. Mean effective radiation dose was 0.39±0.15mSv. Percentages of images with motion artifacts were 0.3% for cystic fibrosis, 1.3% for bronchiolitis obliterans, and 1.1% for congenital pulmonary airways malformations. CONCLUSION: Chest ultra-low-dose computed tomography without sedation or anesthesia delivering a sub-millisievert dose can provide image quality to allow identification of common pulmonary anatomy and diseases.

Case Definition of Chronic Pulmonary Aspergillosis in Resource-Constrained Settings.

Chronic pulmonary aspergillosis (CPA) is a recognized complication of pulmonary tuberculosis (TB). In 2015, the World Health Organization reported 2.2 million new cases of nonbacteriologically confirmed pulmonary TB; some of these patients probably had undiagnosed CPA. In October 2016, the Global Action Fund for Fungal Infections convened an international expert panel to develop a case definition of CPA for resource-constrained settings. This panel defined CPA as illness for >3 months and all of the following: 1) weight loss, persistent cough, and/or hemoptysis; 2) chest images showing progressive cavitary infiltrates and/or a fungal ball and/or pericavitary fibrosis or infiltrates or pleural thickening; and 3) a positive Aspergillus IgG assay result or other evidence of Aspergillus infection. The proposed definition will facilitate advancements in research, practice, and policy in lower- and middle-income countries as well as in resource-constrained settings.

Management of lung nodules in Brazil-assessment of realities, beliefs and attitudes: a study by the Brazilian Society of Thoracic Surgery (SBCT), the Brazilian Thoracic Society (SBPT) and the Brazilian College of Radiology (CBR).

BACKGROUND: Pulmonary nodules are common; some are inconsequential while others are malignant. Management of solitary pulmonary nodule (SPN) in Brazil appears to be highly variable, potentially leading to suboptimal outcomes. Assessment of the variability and the association with the degree of availability of resources can provide a foundation for development of clinical guidelines for management of SPN specific for the Brazilian setting. METHODS: A web-based survey was developed by thoracic surgeons, pulmonologists and radiologists to evaluate SPN perception and management. This survey was sent to their respective national societies members and answers collected between August and December 2016. That included multiple choice questions regarding age, specialty, SPN management, accessibility to exams and interventional procedures characterizing public (SUS) and supplementary private working settings. RESULTS: A total of 461 questionnaires were answered. More than half of participants live in cities with over one million people. Specialties were reasonable equilibrated with 43.5% radiologists, 33.5% thoracic surgeons, 20.3% pulmonologists and 2.6% others. Most of the respondents work in both public and private sector (72.7%). Private has a similar reality compared to well-developed nations regarding exams accessibility and interventions. SUS setting has a significant variability access according to the participants. CT is only easily available in 31.9% of cases, PET-CT is easily available in 24.4%, bronchoscopy is easily available for 42.8%, transthoracic needle biopsy is only easily available in 13.9% and video-assisted thoracoscopic surgery (VATS) biopsy is not available in 19.5%. When there is a probability of malignancy of 50% or higher, 46.5% of participants would be comfortable recommending surgical biopsy. When the probability is higher than 10%, only 36.9% would be comfortable following up radiologically. CONCLUSIONS: Brazil has a very different setting for public and private patients regarding exams accessibility and management options. That might explain why participants have a higher tendency to choose interventional diagnosis and explains why current guidelines may not be applicable to developing countries reality.

Correction to: Multiparametric Magnetic Resonance Imaging in the Assessment of Pulmonary Hypertension: Initial Experience of a One-Stop Study.

The original version of this article unfortunately contained a mistake. There is a typo in the coauthor name, it should be Stephan Altmayer.

Multiparametric Magnetic Resonance Imaging in the Assessment of Pulmonary Hypertension: Initial Experience of a One-Stop Study.

INTRODUCTION: Our goal was to assess the diagnostic performance of magnetic resonance imaging (MRI) as a single method to diagnose pulmonary hypertension (PH) compared to right heart catheterization (RHC), computed tomography (CT), and ventilation/perfusion (V/Q) scintigraphy. METHODS: We identified 35 patients diagnosed with PH by RHC in our institution who have also undergone a CT, a scintigraphy, and an MRI within a month. All cases were discussed in multidisciplinary meetings. We performed correlations between the MRI-derived hemodynamic parameters and those from RHC. The sensitivity and specificity of MRI were determined to identify its diagnostic performance to identify chronic thromboembolic pulmonary hypertension (CTEPH) and interstitial lung disease PH. The gold standard reference for the diagnosis of CTEPH and ILD was based on a review of multimodality imaging (V/Q scintigraphy and CT scan) and clinical findings. RESULTS: Our results showed a good correlation between the hemodynamic parameters of cardiac MRI and RHC. Pulmonary vascular resistance had the best correlation between both methods (r = 0.923). The sensitivity and specificity of MRI to diagnose CTEPH was 100 and 96.8%, respectively. For the ILD-related PH, the MRI yielded a sensitivity of 60.0% and a specificity of 100%. Additionally, cardiac MRI was able to confirm all cases of PAH due to congenital heart disease initially detected by echocardiography. CONCLUSIONS: MRI represents a promising imaging modality as an initial, single-shot study, for patients with suspected PH with the advantages of being non-invasive and having no radiation exposure.

Niemann-Pick disease type B: HRCT assessment of pulmonary involvement / Doença de Niemann-Pick tipo B: avaliação do comprometimento pulmonar por TCAR

ABSTRACT Objective: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. Methods: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen. Results: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone. Conclusions: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.

RESUMO Objetivo: Analisar os achados de TCAR em pacientes com doença de Niemann-Pick (DNP) tipo B a fim de avaliar a frequência dos padrões tomográficos e sua distribuição no parênquima pulmonar, além das características clínicas mais frequentes. Métodos: Foram estudados 13 pacientes (3 do sexo masculino e 10 do sexo feminino) com idades variando de 5 a 56 anos. As imagens de TCAR foram avaliadas por dois observadores de forma independente, e os casos discordantes foram resolvidos por consenso. Os critérios de inclusão foram presença de anormalidades na TCAR e diagnóstico confirmado de DNP tipo B por exame anatomopatológico através de biópsias de medula óssea, pulmão ou fígado. Resultados: Os achados clínicos mais comuns foram hepatoesplenomegalia e dispneia leve a moderada. Os padrões tomográficos mais frequentes foram espessamento liso de septos interlobulares e opacidades em vidro fosco, presentes em todos os pacientes. Linhas intralobulares estiveram presentes em 12 pacientes (92,3%). O padrão de pavimentação em mosaico foi observado em 5 pacientes (38,4%). Áreas de aprisionamento aéreo foram identificadas em 1 dos casos (7,6%). O comprometimento pulmonar foi bilateral em todos os casos, sendo o terço inferior dos pulmões a região mais envolvida. Conclusões: O achado de espessamento liso de septos interlobulares, com ou sem opacidades em vidro fosco associadas, em pacientes com hepatoesplenomegalia é o achado mais frequente na DNP tipo B.

Niemann-Pick disease type B: HRCT assessment of pulmonary involvement.

OBJECTIVE: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. METHODS: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen. RESULTS: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone. CONCLUSIONS: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.