Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach
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...
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doaj-67cc819657e34ae0929701bc19b6a64d2020-11-24T23:15:50ZengWolters Kluwer Medknow PublicationsAnnals of Thoracic Medicine1817-17371998-35572017-01-0112320921210.4103/atm.ATM_47_17Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approachAbdulaziz Uthman JouryAhmad Amer Al BoukaiTarek Seifaw KashourFibrosing 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.http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2017;volume=12;issue=3;spage=209;epage=212;aulast=JouryFibrosingmediastinitispulmonarytherapy |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Abdulaziz Uthman Joury Ahmad Amer Al Boukai Tarek Seifaw Kashour |
spellingShingle |
Abdulaziz Uthman Joury Ahmad Amer Al Boukai Tarek Seifaw Kashour Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach Annals of Thoracic Medicine Fibrosing mediastinitis pulmonary therapy |
author_facet |
Abdulaziz Uthman Joury Ahmad Amer Al Boukai Tarek Seifaw Kashour |
author_sort |
Abdulaziz Uthman Joury |
title |
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach |
title_short |
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach |
title_full |
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach |
title_fullStr |
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach |
title_full_unstemmed |
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach |
title_sort |
severe fibrosing mediastinitis with atypical presentation: effective control with novel therapeutic approach |
publisher |
Wolters Kluwer Medknow Publications |
series |
Annals of Thoracic Medicine |
issn |
1817-1737 1998-3557 |
publishDate |
2017-01-01 |
description |
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. |
topic |
Fibrosing mediastinitis pulmonary therapy |
url |
http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2017;volume=12;issue=3;spage=209;epage=212;aulast=Joury |
work_keys_str_mv |
AT abdulazizuthmanjoury severefibrosingmediastinitiswithatypicalpresentationeffectivecontrolwithnoveltherapeuticapproach AT ahmadameralboukai severefibrosingmediastinitiswithatypicalpresentationeffectivecontrolwithnoveltherapeuticapproach AT tarekseifawkashour severefibrosingmediastinitiswithatypicalpresentationeffectivecontrolwithnoveltherapeuticapproach |
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1725589376514129920 |