致死的石灰化症における多肢壊死。症例報告

Multiple extremity necrosis in fatal calciphylaxis: Case report.

• Diego Ennes Gonzalez
• Renato Demarchi Foresto
• Ana Luiza Santos Maldonado
• Wallace Stwart Carvalho Padilha
• Fernanda Badiani Roberto
• Maria Eduarda Vilanova da Costa Pereira
• Marcelino de Souza Durão Junior
• Aluizio Barbosa Carvalho

抄録

はじめに:

血管石灰化の臨床的影響は、心血管系の罹患率と死亡率の観点から十分に確立されているが、石灰化などの他の臨床症候群は、頻度は低いものの、慢性腎臓病に大きな影響を及ぼす。

INTRODUCTION: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease.

方法:

症例報告は27歳の女性で、3日間足指に両側性の疼痛を訴えていたが、参照部位に小さな壊死部を認めた。彼女は1型糖尿病（25年前）の既往歴があり、慢性腎臓病を患っており、関節リウマチに加えて腹膜透析を受けていた。関節リウマチの増悪により、ワルファリンの使用を開始した際に、静脈カテーテルの合併症による心血管内血栓を伴い、現在の状態に先行して入院していた。虚血は足にまで進行し、両足切断の必要性が生じた。頸動脈も影響を受けていた。血栓性疾患、血管炎、心内膜炎、またはその他の塞栓性疾患の原因を調査し、破棄した。病理検査では皮膚の壊死と表在性軟部に最近の動脈血栓症とMonckeberg's medial calcificationを認めた。ビスホスホネートとチオ硫酸ナトリウムによる治療を開始し，血液透析に切り替え，ワルファリンを未分割ヘパリンに変更した．すべての治療にもかかわらず，4ヶ月の経過で死亡した。

METHODS: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution.

DISCUSSION:

カルシフィラキシーは、重篤な虚血性障害をもたらす稀な微小血管石灰化症候群である。それは慢性腎臓病のミネラルと骨の障害と血管石灰化のプロモーターと阻害剤の間の不均衡が組み合わさった慢性腎臓病に関連した病因を有しており、特にビタミンK拮抗が重要である。

DISCUSSION: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism.

結論:

治療は複雑で有効性の検証が不十分なため、予防戦略が基本となる。

CONCLUSION: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.