Coronary artery inflammation and thrombosis in Wegener's granulomatosis-polyarteritis nodosa overlap syndrome.
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.
Morbini
P
P
Istituto di Anatomia Patologica, IRCCS Policlinico S. Matteo, Pavia, Italy.
Dal Bello
B
B
Arbustini
E
E
D000368:Aged; D000369:Aged, 80 and over; D001772:Blood Cell Count; D001774:Blood Chemical Analysis; D003327:Coronary Disease / Q000209:etiology* / Q000473:pathology; D003328:Coronary Thrombosis / Q000209:etiology* / Q000473:pathology; D003331:Coronary Vessels / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000473:pathology; D006801:Humans; D009206:Myocardium / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000473:pathology