全身性エリテマトーデス患者における大動脈周囲炎とびまん性心内膜下血管炎-ループスフレアか共存疾患か？

Periaortitis and diffuse subendocardial vasculitis in a patient with systemic lupus erythematosus - Lupus flare or a coexisting disease?

• Ourania Gioti
• Evangelos Theotikos
• Argyro Lazarini
• Andreas S Triantafyllis
• Alexia Balanika
• Maria Boutsikou
• Pavlos Tsinivizov
• Antonis Fanouriakis
• Antonia Elezoglou

抄録

全身性エリテマトーデス（SLE）は、幅広い臨床症状を示す異種疾患です。大動脈周囲炎は、SLEを含む他の自己免疫疾患との関連性がある稀な疾患です。これは心筋の低キネシス、駆出率低下、冠動脈造影が正常な患者では疑われるべきである。心血管磁気共鳴画像（CMR）検査はDSVとループス心筋炎を鑑別するために重要であり，鑑別診断に含めるべきである．ここでは、胸膜炎とDSVの両方を呈したSLE既往歴のある女性患者の一例を紹介し、これらの稀な症状に関連した診断上の課題について議論する。

Systemic lupus erythematosus (SLE) is a heterogeneous disease with a broad spectrum of clinical manifestations. Periaortitis is a rare disorder which may manifest isolated or in association with other autoimmune diseases, including SLE. Another rare, yet severe cardiovascular manifestation of lupus is diffuse subendocardial vasculitis (DSV), which should be suspected in patients presenting with myocardial hypokinesis, impaired ejection fraction and normal coronary angiography. Cardiovascular Magnetic Resonance (CMR) imaging is crucial to distinguish between DSV and lupus myocarditis, which should also be included in the differential diagnosis. Herein, we describe a case of a female patient with pre-existing SLE, who presented with both periaortitis and DSV, and discuss the diagnostic challenges associated with these rare manifestations.

© 2020 The Mediterranean Journal of Rheumatology (MJR).