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.
CONCLUSIONS: 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.

UNASSIGNED: This study aims to develop a scoring system for evaluating the degree of pulmonary vascular stenosis in fibrosing mediastinitis (FM).
UNASSIGNED: A retrospective single-centre study was conducted on 56 patients with FM in China between April 2014 and August 2021. The involvement of pulmonary vessels in patients with FM was assessed using dual-phase computed tomography pulmonary angiography, and we found that 85.7% of the patients had both pulmonary artery (PA) and vein (PV) involvement. PA involvement was mainly located proximal to both the upper PA and the bilateral basal trunk levels in the lower lungs. The involvement of the superior PV was more common than that of the inferior PV, and the right inferior PV was the least involved. Most of these lesions exhibited moderate or severe stenosis. Additionally, a scoring system for evaluating the degree of pulmonary vascular stenosis was developed. A correlation analysis revealed a negative correlation between the final pulmonary vascular score and the pulmonary arterial pressure, pulmonary vascular resistance, and maximum tricuspid regurgitation velocity. The calculated score of 17.1 was the best cut-off value for the diagnosis of mild and severe pulmonary hypertension (PH).
UNASSIGNED: We successfully developed a scoring system for pulmonary vascular stenosis that can be used to evaluate the severity of pulmonary vessel involvement and PH. This scoring system may be relevant in the future development of target-based strategies for percutaneous interventions.

Fibrosing mediastinitis is a rare entity generally caused by granulomatous disease. Most cases develop as a complication of histoplasmosis. Association with tuberculosis has been rarely documented. We report the case of 3 young patients who presented with superior vena cava syndrome several years after treated tuberculosis. Biological, radiological, and histological investigations led to the diagnosis of fibrosing mediastinitis.

This case centers on a 76-year-old male experiencing exertional dyspnea and hemoptysis, with a medical history marked by recurrent pulmonary embolism and chronic obstructive pulmonary disease (COPD). Notably, he resides in a histoplasmosis-endemic area. A computed tomography (CT) pulmonary embolism scan revealed notable findings, including an enlarged right lower pulmonary artery, vascular congestion, atelectasis, and a mass exerting pressure on the right lower pulmonary vein. Biopsy results identified the mass as fibrosing mediastinitis, likely attributed to histoplasmosis. A transthoracic echocardiogram indicated right ventricular dilatation, impaired function, and a right ventricular systolic pressure of 63 mm Hg. During right heart catheterization, the patient displayed disparate pulmonary artery wedge pressures (PAWPs) between the right and left sides. This discrepancy was linked to a blunted back wave from the left atrium to the catheter, induced by pulmonary vein compression. Although an infrequent phenomenon, the recorded asymmetry in PAWPs played a crucial role in guiding accurate patient management. The absence of subsequent evaluation of PAWP on the left side could have altered the treatment plan, potentially delaying appropriate patient care. This case emphasizes the necessity of thorough exploration with right heart catheterization when clinical symptoms warrant, highlighting the importance of standardized practices in such procedures.

An uncommon illness known as fibrosing mediastinitis causes the mediastinum to grow excessively thick fibrous tissue. Fungal or idiopathic origins are the most common etiologies of pathology. In an individual suffering from chronic obstructive pulmonary disease (COPD), fibrosing mediastinitis, which resembled a bronchogenic cancer, was identified during anatomopathological examination following mediastinoscopy.

Fibrosing mediastinitis (FM) is a heterogeneous disease characterized by sclerosing fibrosis of mediastinal structures. Pulmonary hypertension (PH) may complicate the course of the disease and can contribute significantly to the morbidity of FM. Due to the rarity and complexity of the disease, evidence-based guidelines are not currently available, and the optimal treatment approach is unknown. Management approaches should be individualized, and current techniques are often unsatisfactory. Here, we present two cases of PH complicating FM that were managed using pulmonary artery vasodilator therapy with excellent hemodynamic response.

Fibrosing mediastinitis (FM), a rare disorder that is further classified as excessive fibrous tissue that occurs within the mediastinum. Classically, presenting with manifestations dependent on where the fibrous tissue is located. In rare instances, compression of pulmonary vasculature can lead to Pulmonary Hypertension. Pulmonary Hypertension- Fibrosing Mediastinitis (PH-FM) represents a rare complication with minimal available data on incidence. In regards to all-cause mortality, no specific data regarding the prognosis of PH-FM exist. With the scarcity of data, this case aids in the advancement of literature due to unique unilateral obstruction and the need for further analysis on our current treatment.

暂无摘要。

BACKGROUND: Fibrosing mediastinitis is a benign but fatal disorder characterized by the proliferation of fibrous tissue in the mediastinum, causing encasement of mediastinal organs and extrinsic compression of adjacent bronchovascular structures. FM-associated pulmonary hypertension (FM-PH) is a serious complication of FM, resulting from the external compression of lung vessels. Pathologic assessment is important for etiologic diagnosis and effective treatment of this disease.
METHODS: A 59-year-old male patient presented at our hospital and was diagnosed with FM-PH. He declined surgical biopsy that is the reference standard for pathologic assessment, in consideration of the potential risks. Therefore, an endobronchial ultrasound examination was performed, which identified the subcarinal lesion. Under ultrasound guidance, four needle aspirations were carried out, followed by one cryobiopsy. Histopathological examination of transbronchial needle aspiration specimens was inconclusive, while samples from cryobiopsy suggested a diagnosis of idiopathic FM. Further immunophenotyping demonstrated the infiltration of lymphocytes, macrophages, and FOXP3-positive cells in FM-PH.
CONCLUSIONS: Mediastinal cryobiopsy might be a novel and safe option for FM-PH patients who are unwilling or unsuitable for surgical procedure.

Fibrosing mediastinitis (FM) is a rare, potentially progressive disease resulting from an idiosyncratic immune response to a variety of stimuli that lead to fibrous infiltration of the mediastinum and possible narrowing of the bronchovascular structures. We report an unusual case of FM in a pediatric patient presenting as myopericarditis and progressing to pericardial thickening and encasement of the mediastinal vascular structures needing surgical intervention. Imaging, including transthoracic echocardiography, cardiac computed tomography, and cardiac magnetic resonance played a crucial role in the diagnosis, assessment, and follow-up. Contrast-enhanced computed tomography can be especially helpful to demonstrate potential findings associated with FM.