Wegener's granulomatosis presenting as mediastinal soft tissue mass invading the tracheal wall.
Wegener's granulomatosis (WG) is a clinicopathologic entity of unknown cause characterised by a necrotising granulomatous vasculitis affecting multiple organs, especially the upper and lower respiratory tracts, lung and kidney. The lung is the most frequently, and sometimes the only involved organ. Single or multiple nodules, with or without cavitation, are the most common pulmonary manifestations in WG, but mediastinal involvement is atypical. The sole tracheal involvement is rare and hilar and mediastinal involvement has been thought not to be part of the clinical feature. We experienced a rare case of WG presenting as paratracheal mediastinal lesions with tracheal wall invasion, which responded dramatically to corticosteroid treatment. We present this case with a review of the literature.
Song
Y H
YH
The Hospital for Rheumatic Diseases, Department of Internal Medicine, Hanyang University Medical Center, Seoul, Korea.
Kim
T H
TH
Lee
I H
IH
Yang
S C
SC
Park
C K
CK
Jang
S J
SJ
Kim
S Y
SY
D000328:Adult; D018450:Disease Progression; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008477:Mediastinal Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D008727:Methotrexate / Q000008:administration & dosage; D011859:Radiography; D013256:Steroids / Q000008:administration & dosage; D014132:Trachea / Q000000981:diagnostic imaging / Q000473:pathology*; D014133:Tracheal Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D016896:Treatment Outcome
D013256:Steroids; D008727:Methotrexate