Idiopathic focal fibrosing mediastinitis with unilateral pulmonary arterial and venous involvement

Pujitha, Vidiyala; Pandey, Niraj Nirmal; Arvind, Balaji; Kumar, Sanjeev

doi:10.1111/jocs.17182

Cited by 2 publications

(2 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In recent years, stenting from within the pulmonary artery to resolve stenosis due to fibrous tissue constriction is the main clinical treatment, which has significantly improved modality and prognosis of PH-FM. , However, it is expensive and has been found to not be ideal for extensive lesions due to the limited length of the stent. Surgical debridement of fibrous tissues for causative treatment is very risky because most FM occurs in the mediastinum, where large vessels such as the aorta and pulmonary arteries are involved. , Our study proposes a new non-invasive therapeutic strategy, radiotargeted therapy, for the etiology of PH-FM, either alone or in combination with stenting from within the pulmonary artery and includes a series of preclinical evaluations of safety and efficacy.…”

Section: Discussionmentioning

confidence: 99%

“…Surgical debridement of fibrous tissues for causative treatment is very risky because most FM occurs in the mediastinum, where large vessels such as the aorta and pulmonary arteries are involved. 47,48 Our study proposes a new non-invasive therapeutic strategy, radiotargeted therapy, for the etiology of PH-FM, either alone or in combination with stenting from within the pulmonary artery and includes a series of preclinical evaluations of safety and efficacy.…”

Section: ■ Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

et al. 2023

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: ■ Discussionmentioning

confidence: 99%

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

et al. 2023

View full text Add to dashboard Cite

show abstract

A retrospective analysis of transudative pleural effusion due to fibrosing mediastinitis

Yu,

An,

et al. 2024

J Cardiothorac Surg

View full text Add to dashboard Cite

Background Pleural effusion caused by fibrosing mediastinitis is rarely reported. This study aimed to summarize the clinical manifestations, diagnosis and treatment of transudative pleural effusion due to fibrosing mediastinitis. Methods Medical records and follow-up data of 7 patients with transudative pleural effusion due to fibrosing mediastinitis in Beijing Chaoyang Hospital between May 2014 and Feb 2018 were retrospectively analyzed. Results These patients included 4 males and 3 females, with an average age of (64 ± 9) years. There were 3 left-sided effusions, 2 right-sided effusions and 2 bilateral effusions. Previous or latent tuberculosis was found in 6 patients. Pulmonary hypertension was indicated by echocardiography in all the 7 patients. Computed tomography pulmonary angiography (CTPA) of all the 7 cases showed increased soft tissue images visible in the mediastinum and bilateral hilus, different degrees of stenosis or occlusion in the pulmonary artery and pulmonary vein. In addition, 4 cases were found of right middle lobe atelectasis with a mediastinal window setting. There was interstitial pulmonary edema on the side of pleural effusion with a lung window setting. All the 7 patients were treated with intermittent drainage of pleural effusion combined with diuretic therapy. Five patients were treated with antituberculosis therapy. Up to now, two patients died of right heart failure and respiratory failure after 2 and 16 months respectively; The remaining 5 patients were still in follow up. Conclusion Fibrosing mediastinitis can lead to pulmonary vein stenosis or occlusion, and thus cause transudative pleural effusion, which can be detected by CTPA. Pulmonary hypertension, long time of cough, and a history of tuberculosis are common in these patients. The common therapy is intermittent drainage of pleural effusion combined with diuretic therapy.

show abstract

Idiopathic focal fibrosing mediastinitis with unilateral pulmonary arterial and venous involvement

Cited by 2 publications

References 3 publications

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

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

A retrospective analysis of transudative pleural effusion due to fibrosing mediastinitis

Contact Info

Product

Resources

About