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IgG4-related disease of pulmonary artery causing pulmonary hypertension
| Content Provider | Semantic Scholar |
|---|---|
| Author | Deng, Hui Zhao, Sheng Yue, Yunlong Liu, Yong Xu, Yali Qian, Jianglin Ma, Xiaorong Gao, Peiliang Yao, Xiaoyan Jiang, Xin Ran Xu, Xiqi Jing, Zhi-Cheng Wang, Yong Pan, Lei Xue, Xinying |
| Copyright Year | 2018 |
| Abstract | IgG4-related disease (IgG4-RD) is recognized as an immune-mediated condition with pathology features of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis, accompanied with or without elevated serum IgG4 concentrations. However, few of pulmonary artery IgG4-RD causing pulmonary hypertension (PH) was reported.The medical records of 3 patients with pulmonary artery IgG4-RD inducing PH were analyzed retrospectively.Imaging findings demonstrated that the lesions of 3 patients located in pulmonary artery, which were initially diagnosed as pulmonary thrombus or malignant tumor. Computed tomography pulmonary angiography (CTPA), ultrasonic cardiogram, and positron emission tomography/computed tomography (PET/CT) didn't support the diagnosis of pulmonary thrombus or malignant tumor. Right heart catheterization (RHC) showed definite PH. Biopsy by right heart catheterization in 2 patients or pneumonectomy in 1 patient confirmed the diagnosis as IgG4-RD. Treated with glucocorticoids and cyclophosphamide or rituximab, 2 patients' IgG4 concentrations declined sharply and the lesions shrunk gradually. Another patient treated with glucocorticoids died of heart failure.IgG4-RD involved pulmonary artery causing PH was rare. A high index of awareness of this disease is required for early diagnosis and treatment. PET/CT might be a valuable approach to distinguish pulmonary artery IgG4-RD from pulmonary thrombus and malignant tumor. |
| Starting Page | 545 |
| Ending Page | 555 |
| Page Count | 11 |
| File Format | PDF HTM / HTML |
| PubMed reference number | 29768335v1 |
| Alternate Webpage(s) | https://doi.org/10.1097/MD.0000000000010698 |
| DOI | 10.1097/MD.0000000000010698 |
| Journal | Medicine |
| Volume Number | 97 |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Bronchiolitis Obliterans Cardiac Catheterization Procedures Catheterization of right heart Cessation of life Cyclophosphamide Early Diagnosis Heart failure Heart valve disease Hypertensive disease Idiopathic Pulmonary Fibrosis IgG4-Related Disease Neoplasms Patients Phlebitis Pneumonectomy Positrons Pulmonary Hypertension Pulmonary artery structure Right side of heart Ultrasonics (sound) X-Ray Computed Tomography angiogram rituximab |
| Content Type | Text |
| Resource Type | Article |
