Beware of the heart: the multiple picture of cardiac involvement in myositis

A 42-yr-old woman with dermatomyositis had two myocardial infarctions, epis odes of acute chest pain and an acute lung oedema. These events were initia lly misinterpreted as atherosclerotic ischaemic heart disease accompanying the autoimmune disease. The lack of improvement of cardiac symptoms with an ti-ischaemic and immunosuppressive drugs indicated other mechanisms. Intrac oronary drug provocation as well as myocardial biopsy revealed a coincidenc e of small-vessel disease and vasospastic angina as a cause for the severe cardiac symptoms. After initiating therapy with high doses of calcium chann el blockers, marked improvement of cardiac symptoms occurred. In the pathog enesis of cardiac involvement in dermatomyositis, two different mechanisms should be considered: inflammatory processes due to dermatomyositis and vas oconstriction caused by an impaired regulation of vascular tone, such as ab normal vessel reactivity or disturbed neuropeptide release. Signs of this g eneralized vasopathy are Raynaud's phenomenon, Prinzmetal's angina and smal l-vessel disease, which can coincide. In patients with severe cardiac sympt oms and autoimmune diseases, Prinzmetal's angina should be excluded by intr acoronary drug provocation using acetylcholine.