1930Occlusive inflammatory coronary microvascular disease in a patient with a complex autoimmune overlap syndrome presenting with malignant ventricular arrhythmias

Abstract

Introduction: Myocardial involvement in patients presenting with complex autoimmune syndromes represent a diagnostic and therapeutic challange. We present a case of occlusive inflammatory coronary microvascular disease in a patient with systemic lupus erithematosus (SLE), anti-phospholipid autoantibodies syndrome (APLS) and Rosai-Dorfman disease (RDD) overlap syndrome. Case report: A 43-year old male was referred to our unit for ventricular tachycardia (VT) ablation. His past medical history was remarkable for a SLE-APLS overlap syndrome (thrombocytopenia, recurrent deep vein thrombosis, nonerosive arthritis, seizure, glomerolonephritis). In 2014 he developed also a multisystemic lymphadenopathy, with a histological diagnosis of RDD. He had no signs or symptoms of myocardial disease up to 2016, when he had his first VT episode. Baseline ECG and echocardiogram were uremarkable. He had also angiographic evidence of normal coronary arteries. Because of refractory recurrent VTs, in 2016 he underwent unsuccessful attempt of endocardial VT ablation (deep substrate within LV apical wall). The patient was continuously suffering from VTs relapes, haemodynamically tolerated, but recurring despite medical therapy. When he was referred to our center, we decided to clarify aetiology before any therapeutic option. A CMR was performed, and showed diffuse T2/STIR hyperintense spots in myocardium and signs of multifocal microvascular occlusion (MVO). To clarify the nature of the disease (granulomas/myocardial involvement in RDD vs. lupic myocarditis vs. microvascular occlusive disease/vasculitis), a CMR-guided endomyocardial biopsy (EMB) was performed, which showed signs of occlusive microvascular inflammatory disease, with ischemia-reperfusion myocardial damage. Despite successful acute substrate modification was obtained by endoepicardial VT ablation, 5 days later a non-clinical VT was inducible, and dualchamber ICD was finally implanted. Since >10,000 n.v. circulating autoantibodies were found, targeted therapy with rituximab was immediately started. Discussion: The case was particularly intriguing since the patient had multiple autoimmune diseases with potential myocardial involvement. CMR excluded masses possibly associated to RDD and identified signs of CMD but could not univocally interpret abnormal findings. EMB biopsy was necessary to exclude granulomatous diseases, lymphocytic lupic myocarditis, and to confirm inflammation of arteriolar wall and ischaemia-reperfusion damage involving myocytes. Conclusion: In patients presenting with cardiac signs and symptoms in the context of multiple overlapping autoimmune diseases potentially involving myocardium, EMB is necessary to identify specific aetiology, to clarify the pathophysiologic of myocyte damage and subsequently to choose an appropriate treatment.