Morbini P, Dal Bello B, Arbustini E
Istituto di Anatomia Patologica, IRCCS Policlinico S. Matteo, Pavia, Italy.
G Ital Cardiol. 1998 Apr;28(4):377-82.
Coronary arteries are frequently involved in systemic arteritis. The inflammatory infiltrate damages the intima and may trigger the occurrence of coronary thrombosis. We report an extreme example of how intimal inflammation in multiple sites of a coronary tree with and without atherosclerosis may trigger coronary thrombosis, in an elderly female patient who died of a clinically unrecognized systemic autoimmune-inflammatory disorder with necrotizing arteritis. The clinical picture was dominated by abdominal symptoms (peritonitis and possible chronic hepatic disease), renal failure and pulmonary X-ray opacities. A precise clinical diagnosis was not formulated, and the patient died of cardiac arrest 15 days after admission. Autopsy showed findings typical of Wegener's granulomatosis and of systemic arteritis with fibrinoid necrosis and multiorgan infarctions. Wegener's granulomatosis-polyarteritis nodosa overlap syndrome was pathologically diagnosed. Although there were no clinical signs of heart involvement, the coronary tree showed inflammation associated with multiple mural and occlusive thrombi. The atypical severe clinical presentation, the short course of the disease and the age of the patient probably contributed to the non proper clinical diagnosis. Old age does not preclude the occurrence of autoimmune disorders, whose course may be dramatically fatal. The abrupt occurrence of a systemic disease with renal failure, hepatomegaly, lung opacities and serositis should prompt analysis to consider these disorders. If properly diagnosed, cardiac involvement should be suspected in autoimmune disorders, even when clinically silent or masked by the systemic clinical picture. In our patient, the role that heart involvement played in the outcome, if any, remains unknown, even though the postmortem pathological identification of coronary mural and occlusive thrombi is generally sufficient to attribute the final cause of death to coronary thrombosis itself.
冠状动脉常累及系统性动脉炎。炎性浸润会损害内膜，并可能引发冠状动脉血栓形成。我们报告了一个极端案例，在一名死于临床未被识别的伴有坏死性动脉炎的系统性自身免疫性炎症性疾病的老年女性患者中，冠状动脉树多个部位存在或不存在动脉粥样硬化的内膜炎症如何引发冠状动脉血栓形成。临床表现以腹部症状（腹膜炎和可能的慢性肝病）、肾衰竭和肺部X线不透光区为主。未做出准确的临床诊断，患者入院15天后死于心脏骤停。尸检显示有韦格纳肉芽肿和系统性动脉炎的典型表现，伴有纤维蛋白样坏死和多器官梗死。病理诊断为韦格纳肉芽肿 - 结节性多动脉炎重叠综合征。尽管没有心脏受累的临床体征，但冠状动脉树显示出与多个壁层和闭塞性血栓相关的炎症。非典型的严重临床表现、疾病的短病程以及患者的年龄可能导致了临床诊断不当。老年并不排除自身免疫性疾病的发生，其病程可能具有致命性。出现伴有肾衰竭、肝肿大、肺部不透光区和浆膜炎的全身性疾病应促使进行分析以考虑这些疾病。如果诊断正确，即使在临床上无症状或被全身性临床表现掩盖的情况下，自身免疫性疾病也应怀疑有心脏受累。在我们的患者中，心脏受累在结局中所起的作用（如果有的话）仍然未知，尽管死后病理鉴定出冠状动脉壁层和闭塞性血栓通常足以将最终死因归因于冠状动脉血栓形成本身。
