Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
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CASE REPORT
Year : 2017  |  Volume : 12  |  Issue : 3  |  Page : 209-212

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


1 Department of Cardiac Sciences, College of Medicine, King Saud University; King Salman Heart Center, King Fahd Medical City, Riyadh, Saudi Arabia
2 Department of Radiology, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia
3 Department of Cardiac Sciences, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia

Correspondence Address:
Tarek Seifaw Kashour
Department of Cardiac Sciences, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh 12372
Saudi Arabia
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DOI: 10.4103/atm.ATM_47_17

PMID: 28808494

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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.


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