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Thoracic Imaging |
1 From Dept of Radiology, Vancouver Hospital and Health Sciences Centre, 855 W 12th Ave, Vancouver, BC, Canada V5Z 1M9 (T.J., N.L.M., Y.C., P.V.K.); Dept of Radiology, Mayo Clinic, Rochester, Minn (T.E.H.); Dept of Radiology, National Kinki Chuo Hospital for Chest Disease, Osaka, Japan (M. Akira); First Dept of Internal Medicine, Kumamoto University School of Medicine, Japan (K.I., M. Ando); Dept of Radiology, Osaka University Medical School (H.N.). Received May 15, 1998; revision requested Jul 13; revision received Aug 8; accepted Oct 26. Address reprint requests to N.L.M.
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Abstract |
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 TOP Abstract IntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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MATERIALS AND METHODS: Thin-section CT scans in 129 patients with histologically proved idiopathic interstitial pneumonia (35 with usual interstitial pneumonia [UIP], 24 with bronchiolitis obliterans organizing pneumonia [BOOP], 23 with desquamative interstitial pneumonia [DIP], 20 with acute interstitial pneumonia [AIP], and 27 with nonspecific interstitial pneumonia and fibrosis [NIPF]) were independently assessed by two observers without knowledge of clinical or histologic data. The observers recorded the abnormalities, diagnosis, and degree of confidence in their diagnosis. Differential diagnosis was limited to the five types of idiopathic interstitial pneumonia.
RESULTS: The two observers made a correct diagnosis, on average, in 74 (57%) cases. On average, the correct diagnosis was made in 25 (71%) cases of UIP, 19 (79%) of BOOP, 14.5 (63%) of DIP, 13 (65%) of AIP, and 2.5 (9%) of NIPF. The two observers made a correct diagnosis with a high degree of confidence in 50 (39%) readings. There was moderate agreement between the observers for the correct diagnosis (
= 0.55) and for the correct diagnosis with a high degree of confidence ( = 0.65).
CONCLUSION: Except for NIPF, the various subtypes of idiopathic interstitial pneumonias often have a characteristic appearance that allows differentiation at thin-section CT.
Index terms: Bronchiolitis obliteratns organizing pneumonia, 60.2191 • Lung, CT, 60.12111, 60.12118 • Pneumonia, acute interstitial, 60.7921 • Pneumonia, desquamative interstitial, 60.793 • Pneumonia, nonspecific interstitial and fibrosis, 60.7922 • Pneumonia, usual interstitial, 60.792
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Introduction |
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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In recent years, two other forms of idiopathic interstitial pneumonia have been described (5,6): acute interstitial pneumonia (AIP) and nonspecific interstitial pneumonia and fibrosis (NIPF; also called nonclassifiable interstitial pneumonia). AIP corresponds most closely to the entity described in 1944 by Hamman and Rich (7,8).
The authors of a number of recent studies (9–14) have described the diagnostic accuracy of computed tomography (CT) with regard to diffuse infiltrative lung diseases. These studies, however, included small numbers of patients with idiopathic interstitial pneumonias other than UIP. Furthermore, although it has been suggested (15–18) that the various idiopathic interstitial pneumonias have characteristic CT findings, to our knowledge there has been no study to assess the value of thin-section CT for help in distinguishing these entities. The aim of this study was to determine whether the five subtypes of idiopathic interstitial pneumonia can be differentiated on the basis of the pattern and distribution of abnormalities at thin-section CT.
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MATERIALS AND METHODS |
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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The study group included 35 patients with UIP, 20 with AIP, 24 with BOOP, 23 with DIP, and 27 with NIPF. The histologic diagnosis was made on the basis of specimens obtained at open lung biopsy in 103 patients, at autopsy in 18 patients with AIP, and at transbronchial biopsy in eight patients with BOOP. Patients with collagen vascular diseases and immunocompromised patients were excluded from the study. Twenty-four of the 129 patients in the current study were included in previous studies (15–24) in which the CT appearances of idiopathic interstitial pneumonias were assessed; these included 13 patients with AIP and 11 with DIP.
All patients underwent thin-section chest CT. The CT scans consisted of 1–3-mm-collimation sections reconstructed with a high-spatial-frequency algorithm. The protocols consisted of thin sections obtained at 1-cm intervals (66 patients), at 1.5-cm intervals (20 patients), or at 2-cm intervals (32 patients), plus three to five thin sections that complemented a complete conventional CT study of chest (11 patients). All CT scans were performed at end inspiration with the patient in the supine position. The CT scans were obtained with a variety of scanners. No intravenous contrast material was used.
The CT scans were randomized and reviewed separately by two independent chest radiologists (Y.C., P.V.K.) who were unaware of any clinical or histologic findings other than the age and sex of the patient. The observers were aware that only five types of idiopathic interstitial pneumonia were included in the study, but they did not know the frequency of the various entities. The observers had not previously participated in any study in which CT findings of interstitial pneumonias were assessed and had not previously been involved in any of the cases.
CT scans were assessed for the presence, extent, and anatomic distribution of intralobular reticular areas of opacity, honeycombing, areas of ground-glass attenuation, consolidation, and nodules. The anatomic distribution was noted to be peribronchovascular if there was a predominance along bronchovascular bundles; peripheral (subpleural), if there was a predominance of abnormalities in the outer third of the lung; dorsal, if there was a predominance in the dependent portion; or random, if there was no predominance. Zonal predominance was assessed as being upper, lower, or random. Upper lung zone predominance was considered to be present when most of the abnormalities were above the level of the tracheal carina; lower zone predominance was considered to be present when most of the abnormalities were below this level.
CT findings were interpreted on the basis of previously published (15-24) data on the CT appearance of the five subtypes of idiopathic interstitial pneumonias, as summarized in Table 1. Each observer subsequently noted the most likely diagnosis in each patient and graded the degree of confidence in their diagnosis as high (level 1) or low (level 2). Agreement between the two observers was assessed by using the statistic (25).
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RESULTS |
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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= 0.55) and for the correct diagnosis with a high degree of confidence ( = 0.65).
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The predominant CT findings of UIP consisted of areas of ground-glass attenuation in 46% of readings, reticular opacities in 33%, honeycombing in 20%, and air-space consolidation in 1%. The predominant CT findings of BOOP were air- space consolidation in 56% of readings, areas of ground-glass attenuation in 29%, nodules in 11%, and intralobular reticular opacities in 4%. The predominant CT findings of DIP were areas of ground-glass attenuation in 96% of readings and air- space consolidation in 4%. The predominant CT findings of AIP were areas of ground-glass attenuation in 75% of readings and air-space consolidation in 25%. The predominant CT findings of NIPF were areas of ground-glass attenuation in 48% of readings, intralobular reticular opacities in 22%, air-space consolidation in 19%, nodules in 7%, and honeycombing in 4%.
The incorrect diagnoses for each disease made by each observer are summarized in Table 4. UIP was most frequently misdiagnosed as DIP by observer 1 (60%) and observer 2 (50%). DIP was most frequently misdiagnosed as UIP by observer 1 (50%) and observer 2 (43%). AIP was most frequently misdiagnosed as NIPF by both observers (50% for each observer). Observer 1 incorrectly diagnosed NIPF as DIP in 13 (52%) cases, as BOOP in eight (32%), and as UIP in four (16%). Observer 2 incorrectly diagnosed NIPF as DIP in 11 (46%) cases, as BOOP in six (25%), as UIP in six (25%), and as AIP in one (4%).
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DISCUSSION |
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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The histologic characteristics of DIP are large numbers of macrophages in the air spaces, mild fibrosis, and relative histologic uniformity from field to field (1,4). The predominant thin-section CT findings of DIP consist of bilateral areas of ground-glass attenuation that mainly involve the middle and lower lung zones (16,20).
The histologic characteristics of BOOP are granulation tissue polyps in the lumina of alveolar ducts and bronchioles, in association with a variable degree of interstitial and air-space infiltration by mononuclear cells and foamy macrophages (4). The characteristic thin-section CT findings consist of bilateral areas of consolidation, which often have a predominantly subpleural or peribronchial distribution (17,21).
The histologic characteristics of AIP are alveolar edema, hyaline membrane formation, and extensive fibroblast proliferation but little mature collagen (4,5). The characteristic thin-section CT findings of AIP consist of extensive bilateral air-space consolidation and patchy or diffuse bilateral areas of ground-glass attenuation (22,23).
NIPF is characterized by varying proportions of interstitial inflammation and fibrosis that are temporally uniform (ie, of similar age, either recent and active or old and relatively quiescent in a given patient) (6). Thin-section CT findings were described in a study (24) with seven patients. The predominant CT manifestations were patchy or diffuse areas of ground-glass attenuation with no zonal predominance.
The authors of a number of studies (9–14) have described the diagnostic accuracy of CT in cases of diffuse infiltrative lung diseases. However, these studies included small numbers of patients with idiopathic interstitial pneumonias except for UIP. In these studies, a correct first-choice diagnosis was made in 77%–89% of cases of UIP, in 11%–78% of cases of BOOP, and in 0%–11% of cases of DIP. In comparison with the results in these previous reports (9–13), the percentage of correct diagnoses in the present study was lower in patients with UIP and higher in patients with BOOP or DIP.
Two reasons may account for the lower percentage of correct diagnoses of UIP in the present study. First, the patient population in the present study was quite different from the populations in the previous studies. The previous studies included more heterogeneous study populations with conditions that were not limited to idiopathic interstitial pneumonias. It is more difficult to distinguish UIP from other idiopathic interstitial pneumonias because these conditions share several CT findings, such as ground-glass attenuation and lower lung and peripheral predominance (15–24).
Second, the patients with UIP in the present study had relatively less severe honeycombing and more extensive areas of ground-glass attenuation at thin-section CT, because this study included only cases proved at surgical biopsy. This excluded typical cases of UIP, because patients with typical UIP seldom undergo lung biopsy. In the present study, therefore, the number of patients with UIP who had peripheral honeycombing as a main CT finding was smaller than the number of patients with atypical findings such as a predominance of extensive areas of ground-glass attenuation. As a consequence, it often was difficult to distinguish between UIP and DIP. Both can manifest with extensive areas of ground-glass attenuation and both show peripheral and lower lung zone predominance at CT (15–18).
In the present study, the lowest diagnostic accuracy was observed in cases of NIPF. Half the cases of NIPF were misdiagnosed as DIP; 25%, as BOOP; and 25%, as UIP. This was not surprising, given that, in histologic terms, NIPF is essentially a diagnosis of exclusion and represents cases of idiopathic interstitial pneumonias that cannot be classified into one of the other subtypes (6,18).
Our study has several limitations. The present study included 13 patients with AIP and 11 with DIP who had also been included in previous studies (16,18,20,22,23). These patients were included to provide a sufficient number of cases of these relatively rare conditions. The findings in these patients were similar to the findings in seven other patients with AIP and in 12 with DIP included in the current study. Furthermore, the analysis was performed by two independent observers who had not previously been involved in any of the cases. Therefore, we believe that the findings are representative of the CT manifestations of these two entities and that the conclusion can be drawn that there is considerable overlap between the CT findings of these two entities and those of other idiopathic interstitial pneumonias.
Because no clinical information was provided, the observers were at an unrealistic disadvantage. In clinical practice, AIP is readily distinguished from the other entities because of its acute onset over days or weeks, whereas UIP usually manifests over months or years (4,5,26,27). It cannot be overemphasized that, in daily practice, the CT findings must be assessed in the appropriate clinical context. For example, a patient who presents with a 1–3-month history of low-grade fever, malaise, and dry cough and who has areas of consolidation is much more likely to have BOOP than any other idiopathic interstitial pneumonia. In this clinical context, the diagnosis of BOOP can be strongly suggested owing to the presence of patchy peribronchovascular and subpleural consolidation. A patient who presents with a 1–3-day history of rapidly progressive shortness of breath and has similar CT findings is more likely to have AIP.
The study is also biased because, in clinical practice, the differential diagnosis must include entities other than the idiopathic interstitial pneumonias. However, the main goal of this study was to determine whether the various idiopathic interstitial pneumonias were associated with findings characteristic enough to allow their distinction at thin-section CT. This goal was achieved, we believe, by having the observers review the findings without knowledge of the clinical data and by committing to a diagnosis on the basis of previous data in the literature. The previous data often were based on review of findings in patients with a known diagnosis and were, therefore, biased toward recognition of expected patterns and distributions of abnormalities.
On the basis of the results of the present study, we consider the most helpful findings in distinguishing the various idiopathic interstitial pneumonias to be (a) the presence of subpleural honeycombing for the diagnosis of UIP, (b) a combination of peripheral consolidation and nodules for the diagnosis of BOOP, (c) extensive consolidation with a random distribution as most suggestive of AIP, and (d) extensive ground-glass attenuation and mild fibrosis as most suggestive of DIP. However, our results show considerable overlap between the CT findings of the various entities, which, in the majority of cases, precludes a confident diagnosis when the diagnosis is established exclusively on the basis of the CT findings.
In conclusion, except for NIPF, the various subtypes of idiopathic interstitial pneumonias included in the current study often can be differentiated on the basis of the pattern and distribution of abnormalities at thin-section CT. However, there is considerable overlap among the CT findings. The CT findings, therefore, must be interpreted in conjunction with the clinical findings. Lung biopsy often is necessary to help establish a definitive diagnosis.
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Footnotes |
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Author contributions: Guarantors of integrity of entire study, T.J., N.L.M.; study concepts, N.L.M.; study design, T.J., N.L.M.; definition of intellectual content, T.J., N.L.M.; literature research, T.J., K.I.; clinical studies, T.J., T.E.H., K.I., M. Akira; experimental studies, T.J., Y.C., P.V.K.; data acquisition, T.J., T.E.H., K.I., M. Ando; data analysis, T.J., N.L.M.; statistical analysis, T.J., N.L.M.; manuscript preparation, T.J., N.L.M.; manuscript editing and review, N.L.M., H.N., M. Ando.
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References |
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TOPAbstractIntroductionMATERIALS AND METHODSRESULTSDISCUSSIONReferences |
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