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

Autor: Abdulaziz Uthman Joury, Ahmad Amer Al Boukai, Tarek Seifaw Kashour
Jazyk: angličtina
Rok vydání: 2017
Předmět:
Zdroj: Annals of Thoracic Medicine, Vol 12, Iss 3, Pp 209-212 (2017)
Druh dokumentu: article
ISSN: 1817-1737
1998-3557
DOI: 10.4103/atm.ATM_47_17
Popis: 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.
Databáze: Directory of Open Access Journals