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

Abstract

A 42-yr-old woman with dermatomyositis had two myocardial infarctions, episodes of acute chest pain and an acute lung oedema. These events were initially misinterpreted as atherosclerotic ischaemic heart disease accompanying the autoimmune disease. The lack of improvement of cardiac symptoms with anti-ischaemic and immunosuppressive drugs indicated other mechanisms. Intracoronary drug provocation as well as myocardial biopsy revealed a coincidence of small-vessel disease and vasospastic angina as a cause for the severe cardiac symptoms. After initiating therapy with high doses of calcium channel blockers, marked improvement of cardiac symptoms occurred. In the pathogenesis of cardiac involvement in dermatomyositis, two different mechanisms should be considered: inflammatory processes due to dermatomyositis and vasoconstriction caused by an impaired regulation of vascular tone, such as abnormal vessel reactivity or disturbed neuropeptide release. Signs of this generalized vasopathy are Raynaud's phenomenon, Prinzmetal's angina and small-vessel disease, which can coincide. In patients with severe cardiac symptoms and autoimmune diseases, Prinzmetal's angina should be excluded by intracoronary drug provocation using acetylcholine.

Is it cardiac involvement mimicking acute myocardial infarction in idiopathic inflammatory myopathy?

“…Cases 2-6 responded well to treatment (9)(10)(11)(12)14). Case 2 suffered recurrent severe chest pain and only added calcium antagonism with amlodipine 5 mg/day markedly improved anginal symptoms requiring no further hospital admissions for angina (14).…”

“…Case 2, 5 and 6 had complaints of chest discomfort or chest pain (9,11,14), while case 4 mainly presented with vomiting followed by syncope (12). Case 1 and 3 did not show any symptoms in the heart (10,13).…”

“…The cases comprised 3 PM and 3 DM patients, including 4 females and 2 males (each of which had DM), and no IBM was reported (9)(10)(11)(12)(13)(14). In cases 2 and 4-6, the course of IIM was long and they were administered prednisolone at various doses, while case 3 had an acute onset.…”

“…Except for case 5, coronary angiography showed small changes in all cases, which may not have been associated with the severe chest symptoms (9)(10)(11)(12)(13)(14). Cases 1 and 2 showed occlusive spasm after intracoronary acetylcholine provocation for vasospasm on coronary angiography (13,14). Coronary angiography of case 5 showed a left anterior descending coronary artery (LAD) dissection from the mid-to-distal part with 95% stenosis (11).…”

“…Case 2 suffered recurrent severe chest pain and only added calcium antagonism with amlodipine 5 mg/day markedly improved anginal symptoms requiring no further hospital admissions for angina (14). Most studies considered the cause of the symptoms to be inflammatory processes due to IIM and vasoconstriction due to impaired regulation of abnormal vasomotion (9,10,(12)(13)(14).…”