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Elevated serum immunoglobulin-G4 (IgG4) has been linked to several overlapping syndromes that are related to IgG4-producing plasma cells. These clinical syndromes include cholangitis, autoimmune hepatitis and pancreatitis, Sjögren's syndrome, nephritis, and retroperitoneal fibrosis, and the spectrum of this disease has been labeled “IgG4-related systemic disease.” Management of IgG4-related systemic disease generally includes immunosuppressive therapy; dramatic responses to medical therapy have obviated surgical management in many cases.
Inflammatory aneurysms have long proved to be challenging for vascular surgeons, with chronic periaortitis, inflammation, and fibrosis increasing the difficulty of surgical repair, and, possibly paradoxically, reducing the risk of rupture. The authors have previous published their examination of a small series (n=10) of inflammatory aneurysms, and showed that 4 of the 10 inflammatory aneurysms had abundant IgG4-positive plasma cells and histological changes typical of IgG4-related systemic disease. In this paper, the authors expand their examination to the 23 cases of inflammatory aneurysms that were treated over 12 years, 13 cases of which contained IgG4-positive plasma cells. These patients were associated with reduced risk of rupture, other allergic or autoimmune disorders, and positive serology for IgG4, IgE, and ANA, compared to the 10 cases that did not contain IgG4-positive plasma cells.
The implications of this report suggest that IgG4-related inflammatory aneurysms may be treated medically, rather than surgically; conversely, the high rate of rupture in IgG4-negative aneurysms suggests an aggressive therapeutic approach. As the only other report of IgG4-related inflammatory aneurysm disease has come from another Japanese group,1 the utility of these observations will be clarified as they are extended to other populations.
There are implications for other vascular diseases as well. For example Kawasaki disease is an acute inflammatory childhood vasculitis with intense secretion of cytokines that permanently damage the vascular endothelium and typically result in coronary aneurysms. This disease is usually treated with immune globulin and aspirin, with steroid therapy being controversial. Should Kawasaki disease prove to be IgG4-related, then additional insights into its management will be obtained.
Ultimately the increasing importance of the role of the immune system in the pathogenesis of atherosclerosis is being recognized. If IgG4-related systemic disease plays a causative role in the formation of atherosclerosis in even a small subset of patients, medical therapy for this important vascular disease may ultimately prove to be a reality.
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