Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography

The accuracies of chest radiography and computed tomography (CT) in the prediction of specific diagnoses in 118 consecutive patients with chronic diffuse infiltrative lung disease (DILD) were compared. The radiographs and CT scans were independently assessed by three observers without knowledge of clinical or pathologic data. The observers listed the three most likely diagnoses in order of probability and recorded the degree of confidence they felt in their first-choice diagnosis on a three-point scale. Confidence level 1 (definite) was reached with 23% of radiographic and 49% of CT scan readings, and the correct diagnosis was made with 77% and 93% of those readings, respectively (P less than .001). The correct first-choice diagnosis regardless of the level of confidence was made with 57% of radiographic and 76% of CT scan readings (P less than .001). The CT scan interpretations were most accurate in silicosis (93%), usual interstitial pneumonia (89%), lymphangitic carcinomatosis (85%), and sarcoidosis (77%). Observers correctly predicted whether a transbronchial or open lung biopsy was indicated with 65% of radiographs and 87% of CT scans (P less than .001). It is recommended that CT be performed before lung biopsy in all patients with chronic DILD.     

Asbestos-related pleural disease and asbestosis: a comparison of CT and chest radiography

High-resolution CT (HRCT) has the ability to demonstrate both asbestos-related pleural disease and parenchymal abnormalities consistent with asbestosis. The role of CT in the diagnosis of asbestosis can be defined by comparing it with radiography. We evaluated 60 men who had a history of occupational exposure to asbestos and whose outside chest radiographs were considered abnormal. Chest radiographs (inside films) and HRCT were performed in all patients at our institution and were interpreted independently by experienced radiologists. Outside film results were compiled from the submitted reports. The final conclusion regarding the interpretation of the radiologic examinations was determined by consensus when disagreements existed. Positive predictive values (the likelihood that a positive report is correct) for pleural disease were: outside films 56%, inside films 79%, HRCT 100%. The positive predictive values for parenchymal disease were: outside films 51%, inside films 83%, HRCT 100%. The addition of HRCT to chest radiography is most useful in eliminating false-positive diagnoses of asbestos-related pleural disease caused by subpleural fat and false-positive diagnoses of parenchymal asbestosis in patients with extensive plaques or emphysema obscuring lung detail. The interpretation of chest radiographs in patients exposed to asbestos is often extremely difficult and subjective, and we recommend that positive findings (except calcified plaques) be confirmed with HRCT.     

Radiology of diffuse interstitial pulmonary disease in children

Diffuse interstitial lung disease (DILD) represents a heterogeneous group of disorders characterised by restrictive lung function and impaired gas exchange. As these diseases occur on a background of the developing lungs and immune system, the clinical presentation and disease progression is modified by comparison with their adult equivalents; thus, often differs markedly in presentation, clinical features and progress from ILD in adults, and it is not safe to extrapolate from adults to children. It is important to understand the normal growth and development of the lungs in children to understand the development of interstitial lung disease. As the chest radiograph is often non-specific, HRCT has been shown in adults and children to increase the accuracy at diagnosis of diffuse lung disease. The trade-off in sensitivity and specificity of HRCT over chest X-ray is related to radiation dose which is significantly higher with conventional spiral or volumetric CT; however, the use of low-dose (50 mA, 0.75 s) limited (1-mm slices every 15-20 mm) HRCT in inspiration with three expiratory supplementary scans allows accurate assessment of the presence and extent of diffuse lung disease at a dose equivalent to approximately ten chest radiographs. Images are reconstructed on a high spatial resolution algorithm and displayed with a wide window setting, at a width of 1500 Hounsfield units (HU) and at a level of -500 HU.     

高分辨力CT在肾移植术后疑似肺炎患者中的应用价值

目的:评价高分辨率CT(HRCT)在诊断肾移植术后疑似肺炎中的应用价值。方法:33例肾移植术后疑似肺炎患者行胸片、胸部HRCT检查。所有患者均经影像学、实验室检查及临床随访而明确诊断。对33例患者的胸片和HRCT图像进行回顾性对比分析。结果:33例中10例(30.3%)胸片未见异常,其中9例HRCT提示肺炎并最终证实;23例胸片和HRCT均见异常,22例最终证实为肺炎,其中18例HRCT可提供更多更准确的信息,仅4例(12.1%)胸片与HRCT及最终诊断结果基本一致。结论:HRCT较胸片更敏感,有助于早期确诊肺炎,所有疑似肺炎的肾移植患者均应行HRCT检查,肾移植术后肺炎常见HRCT改变为磨玻璃样改变、小结节影和小片状实变。     

Radiology of diffuse interstitial pulmonary disease in children

Diffuse interstitial lung disease (DILD) represents a heterogeneous group of disorders characterised by restrictive lung function and impaired gas exchange. As these diseases occur on a background of the developing lungs and immune system, the clinical presentation and disease progression is modified by comparison with their adult equivalents; thus, often differs markedly in presentation, clinical features and progress from ILD in adults, and it is not safe to extrapolate from adults to children. It is important to understand the normal growth and development of the lungs in children to understand the development of interstitial lung disease. As the chest radiograph is often non-specific, HRCT has been shown in adults and children to increase the accuracy at diagnosis of diffuse lung disease. The trade-off in sensitivity and specificity of HRCT over chest X-ray is related to radiation dose which is significantly higher with conventional spiral or volumetric CT; however, the use of low-dose (50 mA, 0.75 s) limited (1-mm slices every 15–20 mm) HRCT in inspiration with three expiratory supplementary scans allows accurate assessment of the presence and extent of diffuse lung disease at a dose equivalent to approximately ten chest radiographs. Images are reconstructed on a high spatial resolution algorithm and displayed with a wide window setting, at a width of 1500 Hounsfield units (HU) and at a level of ?500 HU.     

新生儿支气管肺发育不良的影像学表现(附21例分析)

目的：探讨新生儿支气管肺发育不良的影像学特征，提高对该病的认识。方法：搜集新生儿支气管肺发育不良21例，全部病例均摄胸片，其中18例行螺旋CT扫描，对所有病例的影像学表现进行回顾性分析。结果：21例中，胸片显示两肺野模糊，肺透光度减低．类似肺透明膜样病改变j例，局限性肺气肿4例，弥漫性间质纤维化病变4例。胸片无异常8例。18例CT检查，表现为毛玻璃样病变和实变影5例，多发囊泡状阴影11例，肺间质纤维化改变5例。结论：新生儿支气管肺发育不良的诊断主要根据临床病史及影像学资料。胸片、CT表现虽无特征性，但具有诊断意义，尤其是高分辨率CT能提供更多有价值的征象，有助于本病的诊断。     

Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography

High-resolution computed tomographic (HRCT) scans and chest radiographs were obtained in 23 patients with progressive systemic sclerosis (PSS) to assess the diagnostic merits of HRCT compared with chest radiography in detecting interstitial lung involvement in these patients. HRCT scans showed interstitial disease in 21 patients (91%). The most frequent finding was the so-called subpleural lines, which were demonstrated in 17 patients (74%). Honeycombing was seen in seven patients (30%), while parenchymal bands were seen in six patients (26%). Chest radiographs, on the other hand, showed definite interstitial opacification patterns in only nine patients (39%); six patients (26%) had equivocal reticular areas of attenuation, while eight patients (35%) had normal chest radiographs. Thus, HRCT is much more sensitive than chest radiography when assessing minimal interstitial lung involvement in patients with PSS.     

Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of high-resolution and conventional CT

We compared the accuracies of high-resolution CT (HRCT) and conventional CT in determining the specific diagnoses in 75 consecutive patients with chronic diffuse infiltrative lung disease. Without knowledge of clinical or pathologic data, two reviewers independently assessed three separate sets of CT scans in random order: three HRCT scans, three 10-mm collimation CT scans obtained at the same levels as the HRCT scans, and a complete conventional CT scan. The HRCT scans were obtained at the level of the aortic arch, tracheal carina, and 1 cm above the right hemidiaphragm by using 1.5-mm collimation and a high spatial resolution algorithm. Observers gave the most likely diagnosis along with their degree of diagnostic confidence. The correct diagnosis, irrespective of confidence level, was reached with 71% of the HRCT scans and with 72% of both the corresponding 10-mm and complete conventional CT scans. Confidence level 1 (definite) was reached with 49% of HRCT scans, 31% of corresponding 10-mm scans, and 43% of complete conventional CT examinations; the correct diagnosis was made in 92%, 96%, and 94% of those examinations, respectively. In none of the patients were findings on the limited HRCT scan normal when findings on the conventional CT scan were abnormal. We conclude that in most patients with chronic infiltrative lung disease a specific diagnosis can be made by obtaining a limited number of HRCT scans.     

Chest radiography for general practitioners: scope for change?

In order to derive guidelines for general practitioners on the value of chest radiography we prospectively reviewed all chest radiographs over a 10 month period. Radiographic chest examinations (frontal with or without a lateral view) were performed on 2017 patients in the radiology department or at the chest clinic. Patient details were noted, referring practice and waiting times monitored. The adequacy of the referral letter was assessed, and specific predetermined clinical findings were correlated with the radiographic findings. Results were divided into three groups (normal, abnormal with a clinically unrelated or chronic abnormality, and abnormal with a clinically related abnormality). Of these patients, 1245 (62%) had normal examinations and 460 (23%) had clinically related abnormalities. Abnormalities were rare in the 10-29 year age group, and when reassurance was sought for patients with symptoms of non-specific chest pain or general ill health. Patients presenting with haemoptysis or with symptoms of lower respiratory infection, or of chronic airways disease often had an abnormal radiograph. A clinical diagnosis was indicated or implied in 1664 (82%) and confirmed in 876 (53%). The most frequent reason for referral was for reassurance (618 patients). Guidelines based on age and symptoms should rationalize the use of chest radiography by general practitioners.     

Diagnostic accuracy of thin-section CT and chest radiography of pediatric interstitial lung disease

OBJECTIVE: We assessed the accuracy of thin-section CT and chest radiography to diagnose pediatric interstitial lung disease. MATERIALS AND METHODS: We identified 20 infants, boys, and girls (age range, 1 month to 14 years) with histopathologic confirmation of interstitial lung disease. Six boys and girls without interstitial lung disease were also included. Two observers independently assessed chest radiograph and CT images. The observers stated the most likely diagnosis and a differential diagnosis. We evaluated individual CT features and their distribution. RESULTS: Observers' diagnoses on CT images were correct (first choice or differential) in 66% of observations versus 45% on chest radiographs (p < 0.025). Correct diagnoses were made on first choice in 61% of CT observations versus 34% on chest radiographs (p < 0.005). Observers were confident (versus uncertain) in 42% of the CT observations versus 18% on chest radiographs; of the confident diagnoses made on CT, 91% were correct. CT interpretations were most accurate in the diagnosis of pulmonary alveolar proteinosis, congenital lymphangiectasia, and idiopathic pulmonary hemosiderosis. All healthy patients examined with CT were correctly identified as such. We noted a distinctive CT pattern in three patients with nonspecific interstitial pneumonitis and one patient with desquamative interstitial pneumonitis; the CT pattern consisted of upper zone predominant honeycombing on a background of ground-glass attenuation. CONCLUSION: A higher proportion of pediatric interstitial lung diseases can be diagnosed on thin-section CT than on chest radiographs. In our study, confident and correct diagnoses were made more frequently with CT than with chest radiographs.     

Hypersensitivity pneumonitis: sensitivity of high-resolution CT in a population-based study.

OBJECTIVE. Hypersensitivity pneumonitis refers to a group of pulmonary disorders caused by inhalation of organic or inorganic particulates by sensitized persons. The diagnosis relies on a constellation of findings: exposure to an offending antigen, characteristic signs and symptoms, abnormal chest findings on physical examination, and abnormalities on pulmonary function tests and radiographic evaluation. In population-based studies, the sensitivity of chest radiography for detection of this disease is relatively low. The aim of this study was to determine the sensitivity of high-resolution CT (HRCT) for detection of hypersensitivity pneumonitis diagnosed in a population of swimming-pool employees. SUBJECTS AND METHODS. Thirty-one symptomatic employees of a recreation center who were referred because of possible hypersensitivity pneumonitis were examined by using chest radiography, HRCT, and fiberoptic bronchoscopy with bronchoalveolar lavage and transbronchial biopsy. Hypersensitivity pneumonitis was diagnosed in subjects who had two or more work-related signs or symptoms, abnormal results on transbronchial biopsies, and abnormal lymphocytosis as shown by examination of bronchoalveolar lavage fluid. The chest radiographs and HRCT scans were interpreted by consensus by two observers who were unaware of the clinical diagnosis. RESULTS. Only one of 11 subjects with a diagnosis of hypersensitivity pneumonitis had abnormal findings on a chest radiograph. Five had abnormal HRCT findings. The abnormality in each case consisted of small, poorly defined centrilobular nodules with variable profusion. No subject without the disease had abnormal HRCT findings. Those who had granulomas shown by lung biopsy were more likely to have abnormal HRCT findings than were those who had more subtle histologic abnormalities. CONCLUSION. The sensitivity of HRCT for the detection of hypersensitivity pneumonitis in a population-based study is greater than that of chest radiography. The finding of poorly defined centrilobular nodules on HRCT scans should prompt consideration of this disease.     

Acute and subacute non-infectious lung diseases: usefulness of HRCT for evaluation of activity especially in follow-up

PURPOSE: The purpose of this study was to survey the usefulness of high-resolution CT (HRCT) for the evaluation of activity in acute and subacute non-infectious diffuse infiltrative lung diseases before and after corticosteroid treatment. PATIENTS AND METHODS: Sequential HRCT images and chest radiographs obtained before and after treatment were retrospectively evaluated in 33 patients with acute or subacute noninfectious diffuse infiltrative lung diseases. All these patients were histologically confirmed to have pulmonary Inflammation and to have responded to treatment with corticosteroid. Radiographic and CT scores were correlated with the degree of dyspnea and the results of arterial blood gas analysis using Spearman's rank-correlation coefficient. RESULTS: On follow-up HRCT, the profusion score of areas with increased attenuation was significantly correlated with arterial oxygen tension (PaO2) (p=.003, r=-.53) and the alveolar-arterial oxygen tension difference (AaDO2) (p=.001, r=.57). No other correlation was found after treatment. Nodular and linear opacities were more commonly seen on follow-up chest radiographs and HRCT images than on initial ones. CONCLUSION: HRCT is useful for the evaluation of disease activity in acute and subacute noninfectious infiltrative lung diseases before and after treatment if paying special attention to the profusion of ground-glass attenuation. Even if pretreatment HRCT has not been performed, posttreatment HRCT should be examined.     

Assessment of diffuse infiltrative lung disease: comparison of conventional CT and high-resolution CT

The diagnostic accuracies of conventional computed tomography (CT) and high-resolution CT (HRCT) in examination of the entire lungs were compared prospectively, and the diagnostic utility of a limited number of HRCT sections in the evaluation of diffuse infiltrative lung disease (DILD) was determined in 150 consecutive patients. Conventional 10-mm CT sections were equivalent to 1.2-mm HRCT sections in the recognition of nodules, masses, nodular irregularities of the interfaces, large cystic air spaces, and architectural distortion. Conventional CT was superior to HRCT in the diagnosis of micronodules and infiltrates. However, 15% of micronodules were recognized only on HRCT images because of their small size and low density. Fine bronchial and parenchymal lesions were best seen on HRCT scans. HRCT was the only technique that allowed assessment of the presence of areas of ground-glass attenuation. HRCT improves visualization of small parenchymal structures and allows a confident evaluation of DILD, providing that the entire lungs are studied. Nevertheless, an optimal CT evaluation of micronodular patterns requires additional thick-section CT scans.     

The value of thoracic computed tomography in the detection of recurrent Hodgkin's disease

Fifty chest radiographs and concurrent thoracic computed tomography (CT) scans obtained in a total of 44 patients with 50 separate episodes of suspected recurrent Hodgkin's disease were reviewed. Recurrent disease was present in 18 episodes, involving the mediastinum in 12, the lung parenchyma in five and both mediastinum and lung parenchyma in one. In four episodes, mediastinal recurrence was demonstrated on both the chest radiograph and CT scan. In a further two cases, the chest radiograph appeared normal but CT detected recurrence in the mediastinum. In 20 cases, the mediastinal appearances on chest radiography were suspicious but not diagnostic of recurrence, usually because of previous radiotherapy resulting in residual mediastinal widening. Computed tomography diagnosed recurrent disease which was subsequently proven in seven of these cases. Recurrent disease was suggested by CT in a further case, subsequently shown to be radiation fibrosis. Of the 12 remaining chest radiographs in which the mediastinal assessment was indeterminate, CT was true negative for recurrent disease in 10 cases and was also indeterminate in two. Recurrent disease in the lung parenchyma was demonstrated on the chest radiograph and CT scan on five occasions. There was one incident in which the chest radiograph was normal but CT detected recurrent parenchymal disease. The appearances of the lung parenchyma were indeterminate for recurrent disease on three chest radiographs but CT was helpful in only one case in which radiation change alone was diagnosed. In eight cases the diagnosis of recurrent disease by CT resulted in a decision to initiate treatment. Computed tomography is of value in detecting relapse in patients with suspected recurrent Hodgkin's disease when the chest radiograph is inconclusive, and may enable differentiation of radiation change from recurrent disease in the mediastinum.     

Role and limitations of plain radiography in chest radiology]

Despite the recent development of diagnostic modalities such as CT and MRI, plain radiography continues to play an important role in chest radiology. Chest radiography remains the imaging modality of choice for initial examinations because it offers simplicity, low cost, and a large amount of information. It is important for radiologists to understand normal anatomy and variants simulating disease to avoid false-positive interpretations of chest radiographs and unnecessary CT examinations. It should be noted that many cases can be diagnosed correctly by chest radiographs alone. Only after careful evaluation of an abnormality on chest radiograph should CT examination be performed. We should also be aware that some serious chest diseases, including miliary tuberculosis, can present normal chest radiographs in the early stage. In such patients with respiratory symptoms, even when the chest radiograph is negative, CT examination should be performed promptly to avoid disease progression.     

Pulmonary manifestations of Hodgkin's disease: radiographic and CT findings

The aim of this study was to assess the radiological and CT findings in patients with pulmonary Hodgkin's disease and to analyse to what extent CT provides more diagnostic information. In 37 patients with 41 episodes of pulmonary manifestation of Hodgkin's disease (histological diagnosis: 11, clinical diagnosis: 30) 39 radiographs and 33 CT scans were analysed by two readers in consensus. Pulmonary nodules were recorded in 77% of radiographs (CXR) and 88% of CT scans. Nodules were multiple in 67% (CXR) and 86% (CT) and bilateral in 43% (CXR) and 66% (CT) of cases, respectively. Nodule size ranged from 2 to 100 mm. Of the nodules, 83% at radiography and CT, respectively, were < or =30 mm, and again 83% at radiography and CT, respectively, were irregularly marginated. Diffuse infiltration with and without nodules was less common. With pulmonary manifestations at initial diagnosis of Hodgkin's disease there was always hilar or mediastinal lymphadenopathy. Of 20 episodes, in which radiograph and CT had been obtained within 8 days, CT demonstrated pulmonary involvement when chest radiography was normal in 3 cases and demonstrated more lesions in 12 cases. The typical appearance of pulmonary HD consisted of multiple, irregularly marginated pulmonary nodules. Diffuse infiltration was less common. Computed tomography was superior to radiography not only in characterization of lesions but could also demonstrate pulmonary involvement when the radiograph was normal and should, therefore, be used liberally in addition to radiography.     

Summer-type hypersensitivity pneumonitis: comparison of high-resolution CT and plain radiographic findings.

Summer-type hypersensitivity pneumonitis is an immunologic disease that occurs only in Japan. It is a form of hypersensitivity pneumonitis in which the clinical symptoms appear in the summer and subside spontaneously in mid autumn. The purpose of our study was to determine the CT findings in this condition, to compare the CT findings with those on chest radiographs, and to assess the variations in the CT findings over time. Accordingly, high-resolution CT scans and chest radiographs of 15 patients with summer-type hypersensitivity pneumonitis were retrospectively studied. Seven patients had sequential CT examinations 18-37 days apart. The CT scans and chest radiographs were reviewed by two observers independently. CT findings included diffuse micronodules (n = 15), slightly elevated lung density (n = 13), and patchy air-space consolidation (n = 13). In one patient, the findings on a chest radiograph were normal, while CT showed parenchymal abnormalities. In two cases, follow-up CT showed micronodular abnormalities after findings on the chest radiograph had returned to normal. Our results show that high-resolution CT findings of summer-type hypersensitivity pneumonitis include pulmonary micronodules, increased lung density, and air-space consolidation. High-resolution CT appears to be more useful than plain chest radiographs in the evaluation of pulmonary parenchymal abnormalities in this condition.     

Pediatric diffuse lung disease: diagnosis and classification using high-resolution CT.

OBJECTIVE: Our purpose was to categorize high-resolution CT findings in children with diffuse lung disease and to evaluate the accuracy of diagnoses made using CT. MATERIALS AND METHODS: The chest radiographs and high-resolution CT scans of 20 children (1-16 years old; median, 9 years old) with biopsy-proven chronic diffuse lung diseases were reviewed separately by two independent chest radiologists. Thirteen types of diffuse lung disease were included in the study. Radiographic and CT features were noted, and three choices of diagnosis were recorded, with the confidence level. RESULTS: Diagnoses were made with a high degree of confidence (definite or probable) in 25 of 40 interpretations of CT scans, compared with only five of 40 interpretations of chest radiographs (p < .001). Fourteen (56%) of the 25 confident first-choice diagnoses on CT scans were correct, compared with two (40%) of the five interpretations on chest radiographs. Diseases were classified as belonging to one of five distinct groups on the basis of dominant CT features. Airway disease (n = 5) (bronchiolitis obliterans or bronchocentric granulomatosis) showed geographic hyperlucency on CT. Septal disease (n = 4) (lymphangiomatosis, hemangiomatosis, or microlithiasis) showed septal thickening. Infiltrative lung disease (n = 7) (desquamative interstitial pneumonitis, hypersensitivity pneumonitis, or lymphoid interstitial pneumonitis) showed ground-glass opacity. Air-space disease (n = 3) (aspiration, vasculitis, or bronchiolitis obliterans organizing pneumonia) showed lung consolidation. Langerhans' histiocytosis (n = 1) showed cysts and nodules. Surprisingly little overlap was seen among these groups. CONCLUSION: CT increases the level of diagnostic confidence for pediatric infiltrative lung disease, improves diagnostic accuracy, and provides a useful classification system.     

Tuberculosis: frequency of unusual radiographic findings.

The changing pattern of the radiologic presentation of tuberculosis was noted in a review of 100 consecutive cases of pulmonary tuberculosis. Active tuberculosis was not suggested as a radiologic diagnosis in 43% of the patients. Of the 100 cases, only 66 had characteristics of reactivation tuberculosis (i.e., minimal upper lobe infiltrate or more advanced cavitary disease). Of the other 34 patients, 18 had less usual presentations, making the radiographic diagnosis difficult. The remaining 16 patients had radiographic patterns quite unusual for tuberculosis, including masslike densities resembling carcinoma; chronic infiltrates in a lower lobe; miliary tuberculosis, either superimposed on diffuse interstitial lung disease or associated with a diffuse lung pattern atypical for miliary disease; and normal chest radiograph.     

Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care

AIM: To investigate the clinical range and severity of radiological findings in a cohort of patients with primary ciliary dyskinesia (PCD) receiving tertiary care. MATERIALS AND METHODS: The case notes and clinical test results of 89 children attending the paediatric respiratory disease clinic at our institution were retrospectively analysed. Demographic details including age at diagnosis and common presenting signs and symptoms were studied. Results of chest radiographs, microscopy, and high-resolution computed tomography (HRCT) for quantification of lung damage were analysed. RESULTS: In a cohort of 89 children with PCD, a presentation chest radiograph was available in 62% of patients (n=55), with all but one demonstrating changes of bronchial wall thickening. HRCT of the lungs, available in 26 patients, were scored using the system described by Brody et al. analysing five specific features of lung disease, including bronchiectasis, mucus plugging, peribronchial thickening, parenchymal changes of consolidation, and ground-glass density, and focal air-trapping in each lobe. Peribronchial thickening was observed using HRCT in 25 patients, while 20 patients had bronchiectasis. Severity scores were highest for the middle and the lingular lobes. CONCLUSION: The radiographic findings of the largest reported cohort of patients with PCD are presented, with associated clinical findings. Dextrocardia remains the commonest finding on chest radiography. HRCT demonstrates peribronchial thickening and bronchiectasis, which is most marked in the lower zones. Radiological scoring techniques developed for assessment of cystic fibrosis can also be applied for the assessment of disease severity in this patient population.