The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis
Abstract Background Lymphangioleiomyomatosis (LAM) is a rare disease which is easily misdiagnosed. Vascular endothelial growth factor D (VEGF-D), as the most common biomarker, however, is not so perfect for the diagnosis and severity assessment of LAM. Materials and methods The isobaric tags for rel...
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| Format: | Article |
| Language: | English |
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BMC
2024-01-01
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| Series: | Respiratory Research |
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| Online Access: | https://doi.org/10.1186/s12931-024-02685-6 |
| _version_ | 1797276331935793152 |
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| author | Xixi Song Hui Cai Wenjun Peng Ke Chen Zilinuer Abuduxukuer Yingying Zeng Guiping Zhu Chong Lu Yu Chen Jian Wang Ling Ye Meiling Jin |
| author_facet | Xixi Song Hui Cai Wenjun Peng Ke Chen Zilinuer Abuduxukuer Yingying Zeng Guiping Zhu Chong Lu Yu Chen Jian Wang Ling Ye Meiling Jin |
| author_sort | Xixi Song |
| collection | DOAJ |
| description | Abstract Background Lymphangioleiomyomatosis (LAM) is a rare disease which is easily misdiagnosed. Vascular endothelial growth factor D (VEGF-D), as the most common biomarker, however, is not so perfect for the diagnosis and severity assessment of LAM. Materials and methods The isobaric tags for relative and absolute quantitation (iTRAQ)-based method was used to identify a cytoskeleton protein, moesin. 84 patients with LAM, 44 patients with other cystic lung diseases (OCLDs), and 37 healthy control subjects were recruited for collecting blood samples and clinical data. The levels of moesin in serum were evaluated by ELISA. The relationships of moesin with lymphatic involvement, lung function, and treatment decision were explored in patients with LAM. Results The candidate protein moesin was identified by the proteomics-based bioinformatic analysis. The serum levels of moesin were higher in patients with LAM [219.0 (118.7–260.5) pg/mL] than in patients with OCLDs (125.8 ± 59.9 pg/mL, P < 0.0001) and healthy women [49.6 (35.5–78.9) ng/mL, P < 0.0001]. Moesin had an area under the receiver operator characteristic curve (AUC) of 0.929 for predicting LAM diagnosis compared to healthy women (sensitivity 81.0%, specificity 94.6%). The combination of moesin and VEGF-D made a better prediction in differentiating LAM from OCLDs than moesin or VEGF-D alone. Moreover, elevated levels of moesin were related to lymphatic involvement in patients with LAM. Moesin was found negatively correlated with FEV1%pred, FEV1/FVC, and DLCO%pred (P = 0.0181, r = − 0.3398; P = 0.0067, r = − 0.3863; P = 0.0010, r = − 0.4744). A composite score combining moesin and VEGF-D improved prediction for sirolimus treatment, compared with each biomarker alone. Conclusion Higher levels of moesin in serum may indicate impaired lung function and lymphatic involvement in patients with LAM, suggest a more serious condition, and provide clinical guidance for sirolimus treatment. |
| first_indexed | 2024-03-07T15:26:45Z |
| format | Article |
| id | doaj.art-6a505a0f888d44509a252c8b6454bfb6 |
| institution | Directory Open Access Journal |
| issn | 1465-993X |
| language | English |
| last_indexed | 2024-03-07T15:26:45Z |
| publishDate | 2024-01-01 |
| publisher | BMC |
| record_format | Article |
| series | Respiratory Research |
| spelling | doaj.art-6a505a0f888d44509a252c8b6454bfb62024-03-05T16:39:19ZengBMCRespiratory Research1465-993X2024-01-0125111510.1186/s12931-024-02685-6The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosisXixi Song0Hui Cai1Wenjun Peng2Ke Chen3Zilinuer Abuduxukuer4Yingying Zeng5Guiping Zhu6Chong Lu7Yu Chen8Jian Wang9Ling Ye10Meiling Jin11Department of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Pulmonary and Critical Care Medicine, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Pulmonary and Critical Care Medicine, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityDepartment of Allergy, Zhongshan Hospital, Fudan UniversityAbstract Background Lymphangioleiomyomatosis (LAM) is a rare disease which is easily misdiagnosed. Vascular endothelial growth factor D (VEGF-D), as the most common biomarker, however, is not so perfect for the diagnosis and severity assessment of LAM. Materials and methods The isobaric tags for relative and absolute quantitation (iTRAQ)-based method was used to identify a cytoskeleton protein, moesin. 84 patients with LAM, 44 patients with other cystic lung diseases (OCLDs), and 37 healthy control subjects were recruited for collecting blood samples and clinical data. The levels of moesin in serum were evaluated by ELISA. The relationships of moesin with lymphatic involvement, lung function, and treatment decision were explored in patients with LAM. Results The candidate protein moesin was identified by the proteomics-based bioinformatic analysis. The serum levels of moesin were higher in patients with LAM [219.0 (118.7–260.5) pg/mL] than in patients with OCLDs (125.8 ± 59.9 pg/mL, P < 0.0001) and healthy women [49.6 (35.5–78.9) ng/mL, P < 0.0001]. Moesin had an area under the receiver operator characteristic curve (AUC) of 0.929 for predicting LAM diagnosis compared to healthy women (sensitivity 81.0%, specificity 94.6%). The combination of moesin and VEGF-D made a better prediction in differentiating LAM from OCLDs than moesin or VEGF-D alone. Moreover, elevated levels of moesin were related to lymphatic involvement in patients with LAM. Moesin was found negatively correlated with FEV1%pred, FEV1/FVC, and DLCO%pred (P = 0.0181, r = − 0.3398; P = 0.0067, r = − 0.3863; P = 0.0010, r = − 0.4744). A composite score combining moesin and VEGF-D improved prediction for sirolimus treatment, compared with each biomarker alone. Conclusion Higher levels of moesin in serum may indicate impaired lung function and lymphatic involvement in patients with LAM, suggest a more serious condition, and provide clinical guidance for sirolimus treatment.https://doi.org/10.1186/s12931-024-02685-6LymphangioleiomyomatosisBiomarkerProteomicsMoesinVEGF-DSirolimus |
| spellingShingle | Xixi Song Hui Cai Wenjun Peng Ke Chen Zilinuer Abuduxukuer Yingying Zeng Guiping Zhu Chong Lu Yu Chen Jian Wang Ling Ye Meiling Jin The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis Respiratory Research Lymphangioleiomyomatosis Biomarker Proteomics Moesin VEGF-D Sirolimus |
| title | The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis |
| title_full | The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis |
| title_fullStr | The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis |
| title_full_unstemmed | The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis |
| title_short | The role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis |
| title_sort | role of moesin in diagnosing and assessing severity of lymphangioleiomyomatosis |
| topic | Lymphangioleiomyomatosis Biomarker Proteomics Moesin VEGF-D Sirolimus |
| url | https://doi.org/10.1186/s12931-024-02685-6 |
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