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1
Department of Diagnostic Radiology, Mayo Clinic, 200 First St. S.W.,
Rochester, MN 55905.
2
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN
55905.
Received August 30, 1999;
accepted after revision October 19, 1999.
Address correspondence to K. H. Wittenberg.
Abstract
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MATERIALS AND METHODS. We retrospectively reviewed the radiologic images of 15 patients with biopsy-proven Erdheim-Chester disease. Nine patients had chest radiographic images and CT scans that were available for review. Six men and three women were studied (age range, 25-70 years; mean age, 56 years). Two radiologists interpreted all images by consensus. Lung parenchyma was assessed for the type and distribution of disease. Bronchi, pleurae, hila, and mediastinal and extrathoracic structures were evaluated for abnormalities. Pathologic specimens from all patients were reviewed and correlated with radiologic findings.
RESULTS. Eight of nine patients had thoracic images with abnormal findings. The most common radiographic pattern was reticular interstitial opacities with fissural and interlobular septal thickening. CT revealed regions of ground-glass attenuation and centrilobular nodular opacities. Typically, extrapulmonary findings included pleural effusions (6/8 patients), pericardial fluid or thickening (4/8), and extrathoracic infiltrative soft-tissue masses (4/8).
CONCLUSION. The most common findings of Erdheim-Chester disease with pulmonary involvement include an interstitial process characterized by smooth interlobular septal thickening and centrilobular nodular opacities, fissural thickening, and pleural effusions. On CT, six of nine patients had pericardial fluid and thickening or extrathoracic soft-tissue masses. Such findings are characteristic of Erdheim-Chester disease with pulmonary involvement. Definitive diagnosis requires correlating skeletal findings and lung biopsy findings.
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Many individual case reports have published the radiographic and CT findings of pulmonary Erdheim-Chester disease [10,11,12,13,14,15,16]. To our knowledge, no series has specifically examined the thoracic manifestations of Erdheim-Chester disease. We present the radiologic findings of nine patients with pathologically proven Erdheim-Chester disease with pulmonary involvement.
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All images were reviewed by two radiologists. All chest radiographs and CT scans were assessed for the presence, character, and distribution of thoracic disease. Chest radiographs were evaluated for the extent, character, and distribution of lung parenchymal abnormalities. The presence of effusions, adenopathy, and fissural thickening was also determined, and the presence or absence of interlobular septal thickening (the visualization of Kerley B lines) was assessed. On CT scans, lung parenchyma was evaluated for the presence of lung disease, including smooth interlobular septal thickening, ground-glass attenuation, and nodular and air-space opacities. The presence or absence of a mosaic pattern (areas of variable lung attenuation in a lobular or multilobular distribution [17] was determined. Pleurae were assessed for the presence and laterality of effusions and fissural thickening. Note was made of hilar or mediastinal adenopathy (short axis diameter > 10 mm). Scans were analyzed for the presence of centrilobular nodular structures (tiny nodular opacities in the centrilobular region corresponding to the core structure of secondary pulmonary lobules); if present, the structures were graded according to the extent of distribution: diffuse (>50% of lung parenchyma involved) or occasional (<50%). The pericardium was assessed for thickening and the presence of fluid. All extrathoracic soft tissues were evaluated for evidence of infiltrative soft-tissue masses or stranding.
The pathology specimens of five patients, all open-lung biopsy specimens, were reviewed and correlated with radiologic findings.
In eight of nine patients, chest radiographs revealed a diffuse symmetric reticular interstitial pattern of lung disease (Fig. 1A,1B,1C). In seven of these patients, the abnormal pattern was equally distributed in the upper and lower lobes; one patient had greater involvement in the upper lobes. Six patients had interlobular septal thickening, three did not. No patient had adenopathy. Fissural thickening was noted in eight patients, and two patients had pleural effusions, both were unilateral, one right-sided and one left-sided.
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In eight patients, CT revealed diffuse involvement with smooth symmetric interlobular septal thickening (Fig. 2A,2B). In one patient, CT findings were interpreted as normal. The distribution of abnormality on CT was more variable than on chest radiography: three patients had equal involvement of the upper and lower lobes, four had greater involvement of the upper lobes, and one patient had greater involvement of the lower lobe. Prominent centrilobular nodular opacities were noted in six of nine patients (Fig. 3A,3B). In one patient, the centrilobular nodular opacities were diffuse; in five patients, the opacities were localized. Eight of nine patients had regions of ground-glass attenuation. In seven patients, the ground-glass attenuation was diffuse; in one patient, the attenuation had an apical distribution. Of seven patients with areas of ground-glass attenuation, three revealed a mosaic pattern with regions of relatively normal hyperlucent lung. Ground-glass attenuation probably resulted from infiltrative disease, not airways or vascular causes. This presumption was based on the absence of bronchiectasis or air-trapping on CT with expiratory views or clinical signs of pulmonary arterial hypertension, chronic obstructive pulmonary disease, or thromboembolic disease. One patient had centrilobular emphysematous changes in both lung apices.
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Eight patients had fissural thickening. Four patients had pleural effusions and four did not. Of those with pleural effusions, two were bilateral, one was left-sided, and one was right-sided. Mediastinal adenopathy was observed in one patient.
After evaluating the heart and pericardium, we identified four patients with evidence of pericardial fluid or thickening. After evaluating the extrathoracic soft tissues, we identified three patients with evidence of infiltrative soft-tissue processes or masses: one patient had involvement of the axillary soft tissue and breasts (Fig. 3A,3B), one had perinephric fat stranding, and one had a soft-tissue mass encircling the aorta. Overall, six of nine patients had either pericardial fluid or thickening or evidence of a soft-tissue infiltrative process.
Histologically, all five patients for whom lung biopsy slides were available had characteristic histologic features of Erdheim-Chester disease: interstitial thickening with a striking lymphangitic distribution at low magnification (Fig. 4). At higher magnification, the visceral pleura, interlobular septa, and bronchovascular bundles were expanded by a combination of inflammation and fibrosis. Fibrosis was accompanied by an infiltrate of mononuclear inflammatory cells, including a combination of histiocytes, lymphocytes, and scattered plasma cells. In a study by Egan et al. [18], other histopathologic findings are described in greater detail. Immunohistochemical and electron microscopic examination excluded the possibility of Langerhans' cell histiocytosis or venoocclusive disease.
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Erdheim-Chester disease with pulmonary involvement is uncommon. However, once pulmonary involvement develops, the resulting lung disease significantly contributes to morbidity and mortality. Clinically, dyspnea and cough are the most frequent symptoms. Results of pulmonary function tests often reveal moderate restrictive defects with associated decreases in diffusion capacity. Arterial blood gases are typically normal, but may show a mild-to-moderate hypoxemia. Unlike Langerhans' cell histiocytosis, only one report describes Erdheim-Chester disease with acute pulmonary complications [14]. Radiographic evidence of the disease often precedes clinical symptoms.
In our series, eight of nine patients had characteristic radiographic and CT findings, including symmetric reticular interstitial opacities, smooth interlobular septal and fissural thickening, diffuse multifocal regions of ground-glass attenuation, and centrilobular nodular opacities. Our findings differ from those of other case reports. A study by Remy-Jardin et al. [13] reported smooth thickening of the interlobular septa with large areas of lung destruction, findings not discovered in our patients. A study by Devouassoux et al. [14] reported diffuse interstitial thickening in peripheral areas of the lungs without areas of ground-glass attenuation or centrilobular nodular opacities.
Pathologic correlation revealed that the thickened interlobular septa and centrilobular nodular opacities seen on CT corresponded with the presence of an interstitial infiltration by histiocytes or macrophages forming granulomatous lesions with fibrosis. The peripheral subpleural, peribronchovascular, and interlobular distribution of these lesions help to explain the high-resolution CT findings [13].
To our knowledge, no other disease process would consistently produce this constellation of CT findings (i.e., smooth interlobular septal and fissural thickening; diffuse, multifocal regions of ground-glass attenuation; and centrilobular nodular opacities). Smooth interlobular septal thickening with occasional ground-glass opacification is typical of cardiogenic interstitial edema [19], but centrilobular nodular opacities are unusual. Sarcoidosis and lymphangitic carcinomatosis, other causes of septal thickening, are usually nodular not smooth. Lymphangitic carcinomatosis has a multifocal rather than diffuse tendency. Comparable findings exist in pulmonary venoocclusive disease; however, enlarged central pulmonary arteries are also a finding [20], and centrilobular nodular opacities are an infrequent finding. In diffuse pulmonary lymphangiomatosis, diffuse smooth thickening of the interlobular septa accompany occasional patchy ground-glass attenuation; however, the thickening also affects bronchovascular bundles and diffuse infiltration of the mediastinal fat occurs [21]. The distribution also tends to be central and perihilar. Alveolar proteinosis shows thickened interlobular septa; however, an associated air-space component usually appears, resulting from the presence of proteinaceous material in the alveolar spaces, and centrilobular nodular opacities are not expected. Amyloidosis may accompany interlobular septal thickening or irregular lines; however, associated nodules, honeycombing, or both appear, not centrilobular nodular opacities [22].
The exact pathophysiology of Erdheim-Chester disease is poorly understood. It is characterized by multifocal infiltrations in tissue of lipid-laden macrophages that are intimately dispersed throughout a network of fibrous tissue [23]. As illustrated by some case reports, some patients have pathologic findings suggestive of coexisting Erdheim-Chester disease and Langerhans' cell histiocytosis [16,24,25,26]. The two conditions are probably closely interrelated and histiocytes may actually undergo transformation between the two cell lines. Further studies and ultrastructural characterization may help clarify this issue.
One of our patients had characteristic findings on CT without any evidence of skeletal involvement. Whether this reflects a unique pathologic entity in the lung or whether the patient's pulmonary findings have simply preceded the development of skeletal abnormalities is unclear.
Our retrospectively acquired patient population may represent a select group with relatively advanced disease. This selection bias may be reflected in our CT findings. CT scans obtained during the early stages of Erdheim-Chester disease with pulmonary involvement may not duplicate our findings. In such cases, correlation with any skeletal or additional visceral manifestations of histiocytic infiltration would be helpful. Alternatively, CT can be used to direct open lung biopsy for definitive diagnosis.
The most common findings of Erdheim-Chester disease with pulmonary involvement include an interstitial process characterized by smooth interlobular septal thickening and centrilobular nodular opacities, fissural thickening, and pleural effusions. Although each of these findings is nonspecific, the constellation (though not pathognomonic for Erdheim-Chester disease with pulmonary involvement) is strongly suggestive, especially when observed with the typical skeletal findings.
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