[Abstract] OBJECTIVE: To investigate the CT features of pulmonary lymphangioleiomyomatosis combined with thoracic nodulopathy. METHODS: We retrospectively analyzed the CT and HRCT manifestations of a case of pulmonary lymphangioleiomyomatosis combined with thoracic nodulopathy confirmed by open lung biopsy pathology, and summarized its CT features with the literature. RESULTS: The patient’s CT showed diffusely distributed small thin-walled cystic cavities and cornu nodules in both lungs, as well as bilateral hilar and mediastinal lymph node enlargement. CONCLUSION: The CT manifestation of pulmonary lymphangioleiomyomatosis with thoracic nodular disease has both the CT features of simple pulmonary lymphangioleiomyomatosis and simple thoracic nodular disease, but the mechanism of this disease is not yet understood. Lei Zhidan, Department of Radiology, Henan Provincial People’s Hospital
[Keywords] Lymphangioleiomyomatosis, lung; nodular disease, chest; body layer photography, X-ray computer
CT appearances of pulmonary lymphangioleiomyomatosis combined with thoracic sarcoidosis (a report of one case with literature review)
LEI Zhidan1, GE Yinghui1, TANG Xueyi2
(1. Department of Radiology, 2. Department of Respiration, Henan Provincial People’s Hospital, Zhengzhou 450003, China)
[Abstract] Objective To study the CT features of pulmonary lymphangioleiomyomatosis combined thoracic sarcoidosis. methods The CT and HRCT appearances of one case’s pulmonary lymphangioleiomyomatosis combined thoracic sarcoidosis which were proved by open-lung biopsy were retrospectively analyzed, and Results The patient’s bilateral lung showed diffuse lesions with diffusion of the lung, and it’s CT features were summarized with literature review. The patient’s bilateral lung showed diffuse lesions with small thin walled cyst spaces and miliary nodules on CT scan, and her lymph nodes in bilateral pulmonary hili and Conclusion The CT appearances of pulmonary lymphangioleiomyomatosis combined thoracic sarcoidosis simultaneously The CT appearances of pulmonary lymphangioleiomyomatosis combined with thoracic sarcoidosis simultaneously possess the CT features of simple pulmonary lymphangioleiomyomatosis and simple thoracic sarcoidosis, but it’s mechanism is not clear.
[Key words] Lymphangioleiomyomatosis, pulmonary; Sarcoidosis, thoracic.
Tomography, X-ray computed
Pulmonary lymphangioleiomyomatosis (PLAM) is a rare diffuse interstitial lung disease [1- 6], and the incidence of thoracic sarcoidosis is also low in China [7], so the combined incidence of the two is extremely rare. The author has not seen any relevant literature reports. A patient with pulmonary lymphangioleiomyomatosis combined with thoracic sarcoidosis was treated in our hospital, and the clinical and imaging data were retrospectively analyzed, and the CT features of pulmonary lymphangioleiomyomatosis combined with thoracic sarcoidosis were discussed in the literature to improve the understanding and diagnosis of this disease.
1 Data and methods
1.1 Clinical data The patient, female, 44 years old, an employee of a grain reserve, presented with hemoptysis of bright red color, about 5 ml in volume, without cough, fever and night sweats, for no apparent reason 2 months ago. After treatment with cefotaxime sodium and levofloxacin, he developed intermittent hemoptysis several times with a volume of 5 ml. He was given quadruple anti-tuberculosis for cornified pulmonary tuberculosis, but the effect was poor, so he was transferred to our hospital for further treatment. Signs: normal development, moderate nutrition, no enlargement of superficial lymph nodes, no cyanosis of the lips and mouth, no dry or humid straw hat is heard in both lungs. The lung function examination: normal ventilation function of both lungs, diffusion dysfunction. Bronchoscopy: no fungal, antacid bacilli and tumor cells were found in the brushings, a few columnar cells and phagocytes were seen in the lavage fluid microscopy, and no tumor cells were seen; the bronchial biopsy of the lower lobe of the right lung showed chronic inflammation of the bronchial mucosa, and iron-containing hemocytes and hemorrhage were seen, and the biopsy of the posterior basal segment of the lower lobe of the right lung showed granulomatous inflammation.
1.2 Imaging manifestations Chest X-ray showed increased texture in both lungs, mainly in the middle and lower lung fields, widely distributed corn nodules in both lungs, no significant enlargement of the hilum bilaterally, no widening of the mediastinum, normal thorax and cardiac shadow.
Routine CT examination was performed with GE Light speed Plus 4.0 multilayer spiral CT for chest scanning, with layer thickness of 7.5 mm, pitch factor of 1.5:1, reconstruction interval of 5 mm, standard algorithm reconstruction, matrix 512×512, range from the entrance of the thorax to the base of the lung, and lung window and mediastinal window. The main manifestations were diffusely distributed cornu nodules in both lungs, randomly distributed and distributed around the bronchovascular bundles, and no obvious cystic air spaces were seen; the bilateral hilar and mediastinal lymph nodes were mildly enlarged, distributed in the 2R, 4R, 5, 10R and 10L areas, respectively, and the enlarged lymph nodes were about 1.0-2.0 cm in diameter, and no central necrosis or calcification was seen in the enlarged lymph nodes.
HRCT was performed with a layer thickness of 1.25 mm, layer spacing of 10 mm, and bone algorithm image reconstruction from the level of the aortic arch to the diaphragm, observed with a lung window. The main manifestations were diffuse distribution of small thin-walled cystic air spaces in both lungs, the size of which was about 3-10 mm, and most of them were 6-8 mm in size; diffuse distribution of corn nodules in both lungs, mostly less than 3 mm in diameter, distributed in the subpleural interstitium, lobular septa, intralobular septa and around the bronchial vascular bundles; mild irregular thickening of the lobular septa and bronchial vascular bundles, and bead-like changes.
1.3 Pathological findings An open lung biopsy of the right lower lung showed that: ① multiple epithelioid nodules and multinucleated giant cells were seen in the lung tissue, and no case-like necrotic material was seen in the nodules. (ii) Some of the alveolar wall lymphatic vessels in the lung tissue were surrounded by proliferating smooth muscle in a scattered distribution. ③Immunohistochemistry: HMB45(+), ER(+), PR(+), SMA(+),hexamine silver stain(-), antacid stain(-). The final pathological diagnosis: pulmonary lymphangioleiomyomatosis combined with thoracic nodules.
2 Discussion
2.1 Clinical, pathological and CT features of PLAM PLAM is a female lung disease of unknown origin, which belongs to abnormal proliferation of lymphatic tissue in the lung. It occurs in women of reproductive age between 10 and 50 years old, and its occurrence may be related to estrogen levels or genetic factors [1-6]. Taveira-Dasilva reported [1] a group of cases in which good results were received after the application of high-dose progesterone medication, further suggesting that the occurrence of PLAM is related to estrogen levels. Clinically, dyspnea after activity, recurrent episodes of spontaneous pneumothorax, cough, chest pain, sputum and blood, and celiac pleural fluid are often the main symptoms. Most of the pulmonary functional alterations are hypoxemia due to obstructive ventilation dysfunction and diffusion disorders, while a few may manifest as restrictive or mixed ventilation dysfunction [1-4].PLAM is closely associated with tuberous sclerosis [1. 2] and is often accompanied by a high incidence of extra-pulmonary vascular smooth muscle tumors [3.4]. The main pathological changes are abnormal proliferation of immature smooth muscle cells on the alveolar and bronchial walls, pulmonary vessels and lymphatic vessels, causing obstruction of small airway ventilation, as well as lymphatic vessel smooth muscle proliferation and lymphatic vessel dilation, resulting in a series of secondary changes. As a result of small bronchial narrowing, many small air sacs are formed. They are often divided into two types: cystic and smooth muscle cell type [4. 5].
The CT changes of PLAM are related to the duration of the patient’s disease and the severity of the lesion. They often show diffusely distributed, thin-walled cystic cavities of variable size in both lungs [4. 5. 6], mostly with visible thickening of the interstitium, which may be accompanied by pneumothorax, pleural effusion and extra-pulmonary vascular smooth muscle tumors, usually without interstitial fibrosis and nodular shadowing [6]. Although lymph node enlargement has been reported [4], in the light of most of the literature, I believe that thin-walled cystic air spaces with diffuse distribution in both lungs, diffuse interstitial thickening and concomitant pneumothorax, pleural effusion and extra-pulmonary vascular smooth muscle tumors are typical CT features, and they are not accompanied by interstitial fibrosis, multiple intrapulmonary nodules and thickening of nodular bronchovascular bundles (i.e., “tree bud sign”). ” negative), where thin-walled cystic cavities of variable size diffusely distributed in both lungs are the most important basis for the diagnosis of PLAM [6].
2.2 Clinical, pathological and CT features of thoracic nodular disease Nodular disease is a multisystem granulomatous disease, and thoracic nodules and account for about 90% of cases [8], and its incidence is higher in women [9]. The pathogenesis of nodular disease is largely based on a certain genetic susceptibility [10.11] and is influenced by environmental factors that lead to a series of abnormal immune responses. The early stages of the lesion are often asymptomatic and symptomatic, and as the lesion progresses, 40% to 60% may develop respiratory symptoms, mainly in the form of dry cough, slowly progressive dyspnea, and occasionally bloody sputum. There are often no signs and symptoms, and about 20% of the Velcro umbrella spectrum is coarse and negative. The typical pathology of nodular disease is a non-caseating necrotic granuloma composed of epithelial cells, macrophages, and multinucleated giant cells. The diagnosis is usually confirmed by biopsy of nodular granulation tissue because laboratory tests are often not specific and valid [7-12].
The main features are: (1) enlarged hilar and mediastinal lymph nodes [8. 12], often in the 5, 4R, 10R and 10L regions, and bilateral hilar lymph nodes are often symmetrically enlarged, and the enlarged lymph nodes usually do not compress the trachea and bronchi causing obstructive lesions, and the lymph nodes usually do not calcify or only have limited calcification. (2) The main intrapulmonary HRCT manifestations of nodular disease include: firstly, nodules are the most common signs of nodular disease in the lungs [7. 8], with diameters between 2 and 10 mm, sharp margins, and mostly distributed around lymphatic vessels, i.e., in the subpleural, lobular septum, intralobular septum, and around the lymphatic vessels of the bronchial vascular bundles, which are scattered, widely or diffusely distributed in both lungs. Small nodules may fuse with each other to form large nodules, and the distribution of many small nodules around them is called the “nodal galaxy sign”, and the nodules in the subpleural and lobular septa may form small bead-like changes. In addition, intrapulmonary changes include masses, interstitial thickening, and interstitial fibrosis [7. 8]. (iii) The main manifestations of pleural nodular disease are pleural effusion and pneumothorax [13], but they are not easily diagnosed due to lack of specificity.
2.3 Pathogenesis and CT features of pulmonary lymphangioleiomyomatosis combined with thoracic nodulopathy The above discussion indicates that both PLAM and thoracic nodulopathy are prevalent in females, and the patient in this case was a middle-aged female, so it may be one of the risk factors for the combined occurrence of both diseases. Although the patient has no family history, the occurrence of nodular disease is related to environmental factors and autoimmune factors [10.11], and the positive anti-nuclear antibody in this patient indicates that the occurrence of nodular disease may be related to autoimmune factors. However, further studies on estrogen levels, autoimmunity, and genetics are needed to elucidate the pathogenesis of pulmonary lymphangioleiomyomatosis combined with thoracic nodulosis in a large number of cases.
The CT and HRCT of this patient showed not only the most important sign of PLAM, namely small thin-walled cystic air spaces diffusely distributed in both lungs, but also the most important CT manifestation of thoracic nodular disease in the lungs, hilum and mediastinum, i.e., intrapulmonary nodules diffusely distributed in both lungs, mostly in the subpleural interstitium, lobular septa, intralobular septa and around the bronchial vascular bundles, lobular septa and bronchial vascular bundles. The above three CT manifestations are the most prominent HRCT signs of pulmonary changes in nodular disease [7]; the CT manifestations of enlarged hilar and mediastinal lymph nodes in our patient were mildly enlarged, without central necrosis and calcification, and typically distributed in the 2R, 4R, 5, 10R and 10L regions, which is consistent with the literature [8]. Although it has been reported in the literature [4] that pulmonary lymphangioleiomyomatosis can be accompanied by intrapulmonary nodular shadow, ground glass shadow and signs of lymph node enlargement, the author, by reviewing numerous publications [1_ 6, 14], concluded that: its nodular shadow may be an axial image of thickened interstitium; ground glass shadow may be the result of interstitial smooth muscle cell proliferation interstitial thickening resulting in structural changes of alveoli, small airway stenosis resulting in inadequate air perfusion of air spaces and hemorrhage in The lymph node enlargement may be related to old inflammation, and if its distribution is not consistent with the typical manifestation of nodular disease in the chest, the diagnosis of nodular disease is less likely. Therefore, in the light of the literature, I believe that neither simple PLAM nor simple thoracic nodulopathy can be characterized by both diffusely distributed thin-walled cystic cavities and nodules in the lung and symmetrical hilar and mediastinal lymph node enlargement on CT. In this case, the presence of thin-walled cystic cavities, nodules and lymph node changes at the same time, as well as the typical CT presentation, represent the coexistence of PLAM and thoracic nodular disease, and therefore suggest the diagnosis of pulmonary lymphangioleiomyomatosis combined with thoracic nodular disease.
In conclusion, pulmonary lymphangioleiomyomatosis combined with thoracic nodular disease is an extremely rare thoracic disease with features of both simple PLAM and simple thoracic nodular disease, and although the diagnosis of this disease is difficult, it is possible to suggest the diagnosis of this disease based on the combination of clinical and careful analysis of the imaging features of the patient. However, the pathogenesis of this disease cannot be well understood.
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