Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach

Abstract

Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent with the diagnosis of idiopathic FM. Her disease was associated with complete occlusion of three pulmonary veins and the left main pulmonary artery. The patient was treated with initial high-dose steroids followed by maintenance steroid and methotrexate therapy with very good long-term disease control. Clinical response, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate were used to monitor disease activity and response to therapy.

Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review

“…The clinical presentation of FM varies depending on which of the intrathoracic structures are compromised. Pulmonary, bronchial vasculature, superior vena cava (SVC), and esophageal compression can occur in up to 47%, 26%, 19% and 2% of cases, respectively [1,11]. Symptoms may be gradual in onset; often presenting with progressive shortness of breath and cough.…”

“…Several treatment strategies for FM have been described including medical, surgical and non- surgical interventions. Based on case reports, there are different medical options that have provided significant symptoms relief in idiopathic FM which include steroids, methotrexate and rituximab to target B-cell proliferation in selected patients, especially if they have an underlying autoimmune disease [6,11]. The combination of corticosteroids and methotrexate has been reported to provide resolution of symptoms and improvement of hemodynamic parameters related to pulmonary hypertension [11].…”

“…Based on case reports, there are different medical options that have provided significant symptoms relief in idiopathic FM which include steroids, methotrexate and rituximab to target B-cell proliferation in selected patients, especially if they have an underlying autoimmune disease [6,11]. The combination of corticosteroids and methotrexate has been reported to provide resolution of symptoms and improvement of hemodynamic parameters related to pulmonary hypertension [11]. Since fungal antigens are thought to play a role in the pathogenesis of the disease, antifungal therapy has been used as part of the medical treatment but has not shown efficacy and is not currently recommended [1].…”

Sclerosing Mediastinitis Causing Unilateral Pulmonary Edema Due to Left Atrial and Pulmonary Venous Compression. A Case Report and Literature Review

“…[22] reported complete resolution of the disease in a patient with fibrosing mediastinitis and sclerosing cervicitis treated only with prednisolone. Joury et al [21] describe a case of idiopathic SM, associated with occlusion of three pulmonary veins and the left main pulmonary artery, which was treated with initial high-dose steroids followed by maintenance steroid and methotrexate, with very good long-term disease control. Inoue et al.…”

“…Involvement of the pulmonary arteries [6-9] , the pulmonary veins [10-15] , or the central airways [1,16-20] have also been described. Since complete surgical excision of the lesion is sometimes impossible, an alternative treatment approach may be necessary [2,21-27] .…”