An autopsy case of refractory vasculo-Behçet's disease.

Abstract

Pulmonary vascular involvement in Behçet's disease is a rare complication with a poor prognosis. We present an autopsy case of vasculo-Behçet's disease complicated by pulmonary hemorrhage, possibly caused by rupture of pulmonary artery aneurysms. The patient was treated with a combination of high-dose steroids and pulse cyclophosphamide, but he died from massive hemoptysis. This case highlights the need for potent new therapies for patients with vasculo-Behçet's disease refractory to conventional immunosuppressive therapy, such as a combination of steroids and cyclophosphamide.

• Authors: Shinjiro Kaieda, Yoshiaki Zaizen, Yoriko Nomura, Koki Okabe, Seiyo Honda, Masayoshi Kage, Hiroaki Ida, Tomoaki Hoshino, Takaaki Fukuda
• Journal: Modern rheumatology (Mod Rheumatol) Vol. 25 Issue 2 Pg. 307-11 (Mar 2015) ISSN: 1439-7609 [Electronic] England
• PMID: 24533545 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Behcet Syndrome (complications, pathology)
• Hemorrhage (complications, pathology)
• Humans
• Lung Diseases (complications, pathology)
• Male