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Vasculitis beyond aortitis in IgG4-related disease (IgG4-RD): case report and review of the literature

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Abstract

IgG4-related disease (IgG4-RD) can affect almost every tissue/organ. Vascular involvement in the setting of IgG4-RD is increasingly being recognized with most of the cases pertaining patients with aortitis and/or periaortitis with or without aneurysm formation. However, it is now evident that other vessels like iliac arteries, coronary arteries, carotids, and others can also be affected being often underdiagnosed. Vascular involvement is expressed as vessel wall thickening, while aneurysm formation is also occasionally seen. Although histopathological findings are the cornerstone in the diagnosis of IgG4-RD, imaging modalities like magnetic resonance imaging (MRI) and positron emission tomography (PET) are similarly important when vascular involvement occurs, helping in the mapping of the disease and in identifying other, more accessible to biopsy, affected organs. Inflammation markers like erythrocyte sedimentation rate and C-reactive protein have also been described in IgG4-RD patients with vascular involvement. Herein, we present a case of a middle-aged man with long-term high inflammation markers who eventually diagnosed with IgG4-RD after an ascending aorta aneurysm operation, while a subsequent comprehensive magnetic resonance angiography revealed also involvement of the abdominal aorta and the left subclavian and left common carotid. Moreover, we sought to review the current literature about medium- and large-vessel involvement, beyond the aorta, in patients with IgG4-RD.

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Authors and Affiliations

  1. Rheumatology Unit, First Department of Propaedeutic Internal Medicine, “Laiko” General Hospital, National and Kapodistrian University of Athens, Athens, Greece

    George E. Fragoulis, Gerasimos Evangelatos & Maria G. Tektonidou

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  1. George E. Fragoulis
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  2. Gerasimos Evangelatos
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  3. Maria G. Tektonidou
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GEF and GE: reviewed the literature, drafted manuscript.

MT: treating clinician of the patient, critically revised the manuscript.

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Correspondence to George E. Fragoulis.

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Fragoulis, G.E., Evangelatos, G. & Tektonidou, M.G. Vasculitis beyond aortitis in IgG4-related disease (IgG4-RD): case report and review of the literature. Clin Rheumatol 40, 1167–1173 (2021). https://doi.org/10.1007/s10067-020-05302-1

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