Abstract
A 26-year-old male with a 10-year history of untreated cryptogenic intermittent claudication and toe necrosis presented after a recent acute myocardial infarction that required left anterior descending artery stenting. He was referred to our hospital for the diagnosis and treatment of unexplained premature peripheral artery disease (PAD), left ventricular (LV) dysfunction (LV ejection fraction 23%, diffuse hypokinesis), and juvenile hypertension. An enhanced computed tomography confirmed severe PAD with extensive long segment occlusions of the infrarenal abdominal aorta, bilateral iliac and femoral arteries as well as bilateral renal artery stenosis (Panel A). Aortic magnetic resonance imaging (MRI) demonstrated late gadolinium enhancement (LGE) of the circumferential wall in the descending aorta (Panel B) and occlusive abdominal aorta. F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) displayed diffuse FDG uptake in the LV wall (Panel C). These findings suggested a diffuse inflammatory disorder affecting both large and medium sized arteries as well as the myocardium. Confirmatory selective renal angiography revealed multiple microaneurysms as well as significant renal artery stenosis (Panel D). Endomyocardial biopsy was consistent with an inflammatory dilated cardiomyopathy. Following multidisciplinary discussion, the patient was diagnosed with an unusual overlap of polyarteritis nodosa (PAN) and Takayasu arteritis (TA). He ultimately underwent peripheral bypass surgery (infrarenal aorta to left internal iliac and right common femoral arteries), where an arterial biopsy confirmed both PAN and TA. Treatment with immunosuppressive therapy including corticosteroids and azathioprine significantly improved his quality of life and claudication over a 12-month period.
According to the 2012 Revised International Chapel Hill Consensus Conference nomenclature, 1 large-vessel vasculitis includes TA, while medium-vessel vasculitis includes PAN. It is noteworthy that PAN rarely affects large arteries and TA sometimes involves medium-sized arteries. Yet considering that coronary involvement is more frequent in PAN than TA, and that aortic involvement is more frequent in TA than PAN,2,3 this patient manifested an overlap of these two disorders or an unclassified vasculitis. These images demonstrate systemic cardiovascular inflammation associated with autoimmune diseases in the field of vascular medicine.
‘Images in vascular medicine’ is a regular feature of Vascular Medicine. Readers may submit original, unpublished images related to clinical vascular medicine. Submissions may be sent to: Heather Gornik, Editor in Chief, Vascular Medicine, via the web-based submission system at http://mc.manuscriptcentral.com/vascular-medicine
Footnotes
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The authors received no financial support for the research, authorship, and/or publication of this article.