Hughes Stovin症候群、ベーチェット病のまれな型で、再発性心筋内血栓を呈する

Hughes Stovin Syndrome, a Rare Form of Behcet's Disease Presenting as Recurrent Intracardiac Thrombus.

• Anupama B K
• Casey Tymko
• Rogin Subedi
• Jaswinder Virk
• Debanik Chaudhuri

抄録

Hughes Stovin症候群（HSS）は、末梢静脈血栓症を伴う多発性肺動脈瘤および／または気管支動脈瘤を特徴とする特にまれな疾患であり、Behcet病の心血管疾患の一種であると考えられています。HSSの血栓性症状としての心臓内血栓は珍しい症状であり、診断と治療の課題となっている。ここでは、25歳の男性が右心室内血栓を再発した症例を報告している。この患者は心血栓の再発に伴う心血管系合併症のため、臨床経過中に複数回の心臓手術を必要とした。残念なことに、この患者は1年後に肺動脈瘤破裂に伴う大量喀血で再入院し、左下葉切除術を必要とした。我々の症例は、心臓病変の存在下では増強されるこの潜在的に生命を脅かす症候群に関連した重大な罹患率、合併症、および治療上の課題にも焦点を当てている。

Hughes Stovin syndrome (HSS) is a particularly rare disease characterized by multiple pulmonary artery and/or bronchial artery aneurysms with concomitant peripheral venous thrombosis and is believed to be a cardiovascular variant of Behcet's disease. Intracardiac thrombus occurring as a thrombotic manifestation of HSS is an unusual presentation and represents a challenge in diagnosis and treatment. Here we report a 25-year-old male presenting with recurrent right-sided intracardiac thrombi, in whom pulmonary artery aneurysm was later detected in the clinical course corroborating the diagnosis of HSS and leading to appropriate initiation of immunosuppressive agents. The patient required multiple cardiac surgeries during the clinical course for cardiovascular complications associated with recurrent cardiac thrombus. Unfortunately, the patient was readmitted a year later for massive hemoptysis secondary to pulmonary arterial aneurysm rupture requiring left lower lobectomy. Our case highlights also the significant morbidity, complications, and treatment challenges associated with this potentially life-threatening syndrome, which is intensified in the presence of cardiac involvement.

Copyright © 2020, B K et al.