Into the Heart of Polymyalgia Rheumatica Gianfranco Vitiello, MD,* Giovanni Battista Verrone, MD, † Pier Luigi Stefàno, MD, ‡ Mozghan Fayaz Torshizi, MD,§ Sergio Castellani, MD, PhD,* and Daniele Cammelli, MD* DESCRIPTION In January 2017, a previously healthy nonsmoking 61-year- old man presented to the emergency department with shoulder gir- dle pain that started 10 days before presentation. Electrocardiogram result was consistent with subacute inferolateral myocardial infarc- tion (MI). Troponin I (17.12 mg/L), C-reactive protein (205 mg/L), erythrocyte sedimentation rate (55 mm/h), and fibrinogen (1162 mg/ dL) were highly elevated. Transthoracic echocardiography (TTE) revealed a diffuse left ventricular (LV) hypokinesis (ejection frac- tion [EF] 45%) and mild mitral valve regurgitation. Cardiac cath- eterization revealed a severe stenosis of the right coronary artery with massive thrombosis (Fig. A). Two everolimus-eluting coronary stents were positioned (Fig. B). 18 F-fluorodeoxyglucose positron emission tomography demonstrated 18 F-fluorodeoxyglucose accu- mulation around the shoulders associated with grade 2 uptake of thoracic and abdominal aorta consistent with large-vessel vasculitis (LVV) (Fig. C). Color Doppler ultrasonography (CDUS) of the temporal arteries was negative, but a hypoechoic halo compatible with active vasculitis was demonstrated in subclavian and axillary arteries. A diagnosis of polymyalgia rheumatica (PMR) with LVV was made, and prednisone 75 mg/d with a slow-tapering scheme was started. Complete resolution of the symptoms was confirmed in early February, together with the normalization of C-reactive pro- tein (2.6 mg/L), erythrocyte sedimentation rate (14 mm/h), and fi- brinogen (423 mg/dL). 18 F-fluorodeoxyglucose positron emission tomography (Fig. D) and subclavian and axillary artery CDUS confirmed a complete remission of PMR signs. In late February, TTE follow-up revealed thinning of the LV wall (2.9 mm) together with diskinetic wall motion consistent with LV pseudoaneurysm and severe mitral regurgitation. Cardiac mag- netic resonance imaging showed a rim of delayed LVenhancement of the inferolateral wall, parietal thrombotic apposition, and an EF of 36% compatible with pseudoaneurysm (Fig. E). In March, com- plete dissection of the heart was performed, and pseudoaneurysm was closed with sutures reinforced by Teflon felt. A following TTE registered a 58% EF. Control cardiac magnetic resonance im- aging is shown in Figure F. Up to 60% of PMR patients exhibit a concomitant LVV, espe- cially when persistent lack of improvement after steroid therapy and constitutional symptoms are seen. 1 A recent meta-analysis demonstrated a significant increased risk of coronary artery dis- ease (CAD) among PMR patients with a calculated risk ratio of 1.72, even though a link between CAD and LVV has not been demonstrated. 2 Our patient presented an overall low cardiovascu- lar risk because of negative personal and family history for CAD, normal body mass index, and lipid profile. Early-stage diabetes mellitus had only a small role in this acute event, as supported by the absence of diabetes mellitus–related complications. Con- versely, LVVand rheumatic diseases are well-known high-risk fac- tors for CAD, as chronic inflammation leads to coagulation cascade promotion and anticoagulation pathway inhibition. 3 In our patient, acute-onset LVV PMR may have facilitated the throm- botic apposition of the right coronary artery with the consequence of MI. Left ventricular pseudoaneurysms are a rare complication of MI with unknown natural history and elevated surgical mortality rates. 4 In our case, prompt surgical treatment was followed by a complete recovery with conserved EF. For these reasons, identifi- cation of vascular risk factors and careful follow-up aimed at re- ducing this excess risk are recommended, even in patients with apparently low cardiovascular risk. ACKNOWLEDGMENTS The authors thank Prof. Paola Parronchi for comments that greatly improved the article. They also thank Dr. Marzio Taddei for his kind collaboration. REFERENCES 1. Lavado-Pérez C, Martínez-Rodríguez I, Martínez-Amador N, et al. (18)F-FDG PET/CT for the detection of large vessel vasculitis in patients with polymyalgia rheumatica. Rev Esp Med Nucl Imagen Mol. 2015;34: 275–281. 2. Ungprasert P, Koster MJ, Warrington KJ. Coronary artery disease in giant cell arteritis: a systematic review and meta-analysis. Semin Arthritis Rheum. 2015;44:586–591. 3. Esmon CT. The interactions between inflammation and coagulation. Br J Haematol. 2005;131:417–430. 4. Atik FA, Navia JL, Vega PR, et al. Surgical treatment of postinfarction left ventricular pseudoaneurysm. Ann Thorac Surg. 2007;83:526–531. From the *Experimental and Clinical Medicine Department and †Experimental and Clinical Biomedical Sciences Department, University of Florence; and ‡Heart Surgery Unit, Careggi University Hospital, Florence; and §Istituto Radiologico Toscano–Alliance Medical, Pistoia, Italy. The authors declare no conflict of interest. Correspondence: Gianfranco Vitiello, MD, Largo Brambilla 3, Experimental and Clinical Medicine Department, University of Florence, 50100 Florence, Italy. E‐mail: gianfranco.vitiello@unifi.it. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 1076-1608 DOI: 10.1097/RHU.0000000000001049 IMAGES JCR: Journal of Clinical Rheumatology • Volume 00, Number 00, Month 2019 www.jclinrheum.com 1 Copyright © 2019 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.