Pleuro-pericarditis, vasculitis, subendocardial and nodular biventricular fibrosis. The multiple faces of systemic sclerosis detected by cardiac magnetic resonance in the same patient

Abstract

A 60-year old woman with early diffuse systemic sclerosis(SSc) (disease duration 2 years, positive anti-topoisomerase I an-tibodies, megaesophagus, arrythmias and lungfibrosis with PA=30 mm Hg), under treatment with steroids, cyclophosphamide,amiodarone, domperidone, ACE inhibitors and protease pump in-hibitors, presented with recent onset of dyspnoea.The patient was referred to cardiac magnetic resonance (CMR)for evaluation of systolic function of right (RV), left ventricle (LV),myocardial perfusion and presence of myocardial scar. Cine CMR,using steady state free precession sequence (SSFP), applied for functionassessment, detected pleuro-pericarditis, RV dilatation with RVEDV=229.32 ml, RVESV=152.54 ml, RVEF=33.48% and LVEDV=138.17 ml,LVESV=66.81 ml, LVEF=52.37% ( Fig. 1A). For evaluation of myocardialperfusion, the first-pass perfusion imaging (FPP), using gadolinium ‐DTPA (Gd-DTPA), documented a subendocardial hypoenhancementcharacterized by a darker appearance compared to normal myocardium,whichisthetypicalmorphologicalfinding of autoimmune vasculitis(Fig. 1B) [1]. The evaluation of scar tissue was performed from the lategadolinium enhanced (LGE) images, taken 15 min after the Gd-DTPAinjection and using an inversion recovery (IR) sequence. LGE imagesrevealed the coexistence of bo th subendocardial and nodular fibrosis inLV and also free wall RV fibrosis (Fig. 2A, B).SScisamulti-system,connectivetissuediseaseofunknownetiology,characterized by vascular dysfunction, autoimmunity, and enhanced fi-broblast activity resulting in fibrosis of internal organs that conducts toincreased mortality. Primary myocardial involvement is common inSSc and has been recognized as a poor prognostic factor, when clinicallyevident. Recent data strongly suggest that the myocardial involvementisrelatedtocontinuouslyrepeatedepisodesoffocalischemia,duetomi-crocirculation impairment with abnormal vasoreactivity that finallyleadstodevelopmentofirreversiblemyocardial fibrosisandLV–RVdys-function [2,3]. Recentquantitative methods,such as perfusion magneticresonance imaging, have underlined these results [2,3]. Myocardial fi-brosis has been detected by CMR, as a mainly linear and, more rarely,nodular lesion. It is usually mid-wall and, more rarely, subendocardialor subepicardial. Usually, it predominates in mid-anterior, mid-anteroseptal and mid-inferoseptal segment and has no correlation