IGG4 Disease And Slerosing Aortitis

IgG4-related disease (IgG4-RD) is an immune- -mediated fibro-inflammatory condition with unknown etiology that can affect various organs. Although its prevalence is still unknown, it appears to be more frequent in adult males. Cardiovascular manifestations are rare and can include idiopathic retroperitoneal periaortic fibrosis, inflammatory aortic aneurism, inflammatory periarteritis and inflammatory pericarditis. Vascular involvement is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease. The gold standard diagnosis is histological.

A 47-year-old man presented rupture of two aortic aneurysms: one thoracic and one abdominal, and underwent surgical correction. A segment of the aorta artery wall measuring 3x2x0.5cm, exhibited smooth intimate and white vinous adventitia, medium tunic was white, through firm tissue with loss of elasticity.

In addition to heterogenous collagenation with destruction of the elastic network of the aortic mediae tunica, there was fibrin deposition and neutrophil overlap. Lymphoid follicles with reactive germinate centers were along the tunica media and adventitia, without phenotype of endothelitis and absence of either macrophages and Langerhans cells (CD1a). Plasmocytes showed immunopositivity to IgG4, with heterogeneous and well defined localization, supporting the diagnosis of igG4 Disease. Serological studies showed negativity for vasculitis, hereditary connective tissue diseases were not component of the clinical set and normal serum IgG4 concentration was determined.

Few cases of involvement of large vessels by IgG4- -RD have been reported in literature. Serum IgG4 concentration may be normal in one third of patients. In this case, IgG4 immunostaining was crucial for the diagnosis of IgG4-RD aortitis, together with the hyaline destruction of the tunica media (ESP 2015 / Aagaimy 2013). There is still no clinical knowledge for the treatment and monitoring of the involvement of large vessels by IgG4-RD.