Idiopathic fibrous mediastinitis with refractory pleural effusion: a case report and literature review

Dong, Shuangxia; Dai, Xinjian; Jiang, Yi; Zheng, Jiyang

doi:10.1177/03000605211040264

Cited by 3 publications

(3 citation statements)

References 22 publications

(43 reference statements)

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“…[32] FM is a rare condition characterized by proliferation of fibrous tissue in the mediastinum; this leads to pulmonary vascular stenosis owing to wrapping, infiltration, and compression, mostly involving the proximal pulmonary artery. [8,33] PPS typically presents as stenosis, or obstruction a segment of the pulmonary artery tree, and is a congenital or acquired disease. [10,11,34]…”

Section: Pathophysiological Mechanisms Of Cpasinduced Phmentioning

confidence: 99%

Chronic Pulmonary Artery Stenosis: Time to Think as a Disease Entity

Dong,

Zhang,

et al. 2023

Cardiology Discovery

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Chronic pulmonary artery stenosis (CPAS) is characterized by a reduction or complete obstruction of the cross-sectional area of the pulmonary artery owing to various causes. The condition exhibits similar pathophysiological progress, leading to pulmonary hypertension (PH), reduced physical endurance, right heart failure, and death. Although CPAS is often regarded as a subgroup of PH, it can manifest independently for an extended duration before the onset of PH and can significantly impact patient quality of life. It may therefore be more appropriate to consider PH as pathophysiological progression of CPAS, thereby recognizing CPAS as a distinct disease entity.

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Section: Pathophysiological Mechanisms Of Cpasinduced Phmentioning

confidence: 99%

Chronic Pulmonary Artery Stenosis: Time to Think as a Disease Entity

Dong,

Zhang,

et al. 2023

Cardiology Discovery

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“…Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is a benign proliferative disease of fibrous tissues within the mediastinum . Of note, FM can lead to pulmonary hypertension (PH), which is attributed to the wrapping, infiltration, and constriction of large blood vessels and bronchi in terms of mechanisms.…”

Section: Introductionmentioning

confidence: 99%

“…Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is a benign proliferative disease of fibrous tissues within the mediastinum. 1 Of note, FM can lead to pulmonary hypertension (PH), which is attributed to the wrapping, infiltration, and constriction of large blood vessels and bronchi in terms of mechanisms. Importantly, PH has been considered as a major cause of death for FM patients, which is a rare subcategory of PH, and draws more attention from researchers thereby.…”

Section: ■ Introductionmentioning

confidence: 99%

Potential Value of FAPI PET/CT in the Detection and Treatment of Fibrosing Mediastinitis: Preclinical and Pilot Clinical Investigation

et al. 2023

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Fibrosing mediastinitis (FM) is a rare proliferative disease within the mediastinum that leads to pulmonary hypertension, which has been regarded as a major cause of death. This study aims to evaluate the potential value of fibroblast activation protein inhibitor (FAPI)-PET/CT in the integration of diagnosis and treatment of FM through targeting FAPI in fibrosis rats and provide a theoretical basis for clinical management of FM patients. By performing a 18 F-FAPI PET/CT scan, the presence of FAPIavid in the fibrotic lesion was determined. Through a fibrosis rat model, 18 F-FAPI-74 was used for lesion imaging and 177 Lu-FAPI-46 was utilized to investigate the potential therapeutic effect on FM in vivo. In addition, biodistribution analysis and radiation dosimetry were carried out. With the 177 Lu-FAPI-46 pharmacokinetic data of rats as the input, the estimated dose for female adults was computed, which can provide some useful information for the safe application of radiolabeled FAPI in the detection and treatment of FM in patients. Then, major findings on the use of FAPI PET/CT and SPECT/CT in FM were presented. 18 F-FAPI-74 showed a high-level uptake in FM lesions of patients (SUVmax 7.94 ± 0.26), which was also observed in fibrosis rats (SUVmax 2.11 ± 0.23). Consistently, SPECT/CT imaging of fibrosis rats also revealed that 177 Lu-FAPI-46-avid was active for up to 60 h in fibrotic lesions. In addition to this robust diagnostic performance, a possible therapeutic impact was evaluated as well. It turned out that no spontaneous healing of lesions was observed in the control group, whereas there was complete healing on day 9, day 11, and day 14 in the 30, 100, and 300 MBq groups, respectively. With a significant difference in the free of event rate in the Kaplan−Meier curve among four groups (P < 0.001), a dose of 300 MBq displayed the best therapeutic effect, and no obvious damage was observed in the kidney. Furthermore, organ-absorbed doses and an effective dose (0.4320 mSv/MBq) of 177 Lu-FAPI-46 presumed for patients were assumed to give a preliminary indication of its safe use in clinical practice. In conclusion, 18 F-FAPI-46 PET/CT can be a potentially valuable tool for the diagnosis of FM. Of note, 177 Lu-FAPI-46 may be a novel and safe radiolabeled reagent for the integration of diagnosis and treatment of FM.

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Budesonide/formoterol

2022

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Idiopathic fibrous mediastinitis with refractory pleural effusion: a case report and literature review

Cited by 3 publications

References 22 publications

Chronic Pulmonary Artery Stenosis: Time to Think as a Disease Entity

Chronic Pulmonary Artery Stenosis: Time to Think as a Disease Entity

Potential Value of FAPI PET/CT in the Detection and Treatment of Fibrosing Mediastinitis: Preclinical and Pilot Clinical Investigation

Budesonide/formoterol

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