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      Segmental Arterial Mediolysis with Preceding Symptoms Resembling Viral Infection Hampers the Differentiation from Polyarteritis Nodosa

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          Abstract

          A middle-aged man presented with a fever, arthralgia, gastrointestinal symptoms, headache, and rash. After two weeks, the patient suddenly complained of severe abdominal pain, and computed tomography revealed aneurysms in the hepatic and splenic arteries, which increased in size progressively. Given the elevated levels of inflammatory markers and orchitis, polyarteritis nodosa (PN) was initially suspected. Catheter embolization for the ruptured hepatic aneurysm and splenectomy for the large splenic ones were performed, and the pathological finding was consistent with segmental arterial mediolysis (SAM). Changes in inflammatory marker levels and aneurysmal size are also informative to differentiate SAM from PN.

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          Most cited references 20

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          Segmental arterial mediolysis: course, sequelae, prognosis, and pathologic-radiologic correlation.

          Segmental arterial mediolysis is a vascular disease of putative vasospastic origin that causes massive hemorrhages. Although once considered rare, awareness of this disease has resulted in increased reports in the pathology and radiology literature. Despite this, uncertainties concerning pathologic and radiologic correlations, the course of this disease, and aspects of its prognosis exist. This article addresses these issues. Thirteen radiologic reports of segmental arterial mediolysis are analyzed, and slides of 25 cases of segmental arterial mediolysis are searched for lesions analogous to the radiologic findings. Six angiographic presentations are identified: (a) arterial dilatation, (b) single aneurysm, (c) multiple aneurysms, (d) dissecting hematomas, (e) arterial stenosis, and (f) arterial occlusions. Pathologic correlations reveal that lytic loss of medial muscle causes arterial dilatation, dilated arterial gaps form aneurysms, dissections develop at arterial-medial gap junctions or from reparative granulation tissue and reparative alterations, and thrombi cause stenosis and occlusions. The most common radiologic findings at onset are aneurysms, arterial dilatation, and occlusions, while dissections and stenotic lesions often are delayed. These images correlate with the histologic evolution of segmental arterial mediolysis. Segmental arterial mediolysis is an acute limited disease. Sequelae recognized radiologically include aneurysms, dissecting hematomas, arterial stenosis, and occlusions. Generally, these persist, become smaller, or resolve, but symptomatic dissections with delayed onset occur. Sequelae of subclinical forms of segmental arterial mediolysis may cause isolated idiopathic aneurysms or may evolve into arterial lesions indistinguishable from fibromuscular dysplasia.
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            Segmental arterial mediolysis: a systematic review of 85 cases.

            Segmental arterial mediolysis (SAM) is a nonatherosclerotic, noninflammatory arteriopathy of unknown etiology with life-threatening manifestations. With advances in endovascular techniques, SAM is increasingly being managed without the need for major surgery.
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              Segmental arterial mediolysis: CTA findings at presentation and follow-up.

              Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries. The purpose of our study was to retrospectively describe the CT angiography (CTA) findings of this disease and the evolution of those findings over time in five patients. Comparison of CTA and digital subtraction angiography suggests that CTA is useful to diagnose symptomatic segmental arterial mediolysis. Midterm CTA follow-up (median, 3 years) indicates that segmental arterial mediolysis lesions may resolve or remain unchanged.
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                Author and article information

                Journal
                Intern Med
                Intern. Med
                Internal Medicine
                The Japanese Society of Internal Medicine
                0918-2918
                1349-7235
                15 September 2019
                : 58
                : 18
                : 2721-2726
                Affiliations
                [1 ]Department of Rheumatology and Allergology, Shimane Prefectural Central Hospital, Japan
                [2 ]Department of General Medicine, Shimane Prefectural Central Hospital, Japan
                Author notes

                Correspondence to Dr. Norihiro Nagamura, nagamura@ 123456spch.izumo.shimane.jp

                Article
                10.2169/internalmedicine.2487-18
                6794176
                31527370
                Copyright © 2019 by The Japanese Society of Internal Medicine

                The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit ( https://creativecommons.org/licenses/by-nc-nd/4.0/).

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