Correspondence

Ruptured Abdominal Aortic Aneurysm Related to IgG4 Periaortitis

N Engl J Med 2009; 361:1121-1123September 10, 2009

Article

To the Editor:

Periaortitis that is associated with immunoglobulin G4 (IgG4) plasma-cell infiltration is a rare condition with undefined incidence and clinical features. In a single-center study in Japan, the condition accounted for approximately 5% of all surgical abdominal aortic aneurysms and was depicted as generally asymptomatic and without rupture.1

A 63-year-old white woman with history of controlled diabetes, hypertension, and coronary artery disease and without any other systemic or inflammatory disease was admitted to the hospital because of recent-onset severe, sharp abdominal pain. During the previous 6 months, she had had mild-to-moderate lower back pain, decreased appetite, and weight loss of approximately 15%. Three weeks before admission, pain also developed in the lower abdomen; this pain acutely worsened on the day of admission and was associated with light-headedness.

Inpatient evaluation revealed anemia and a multilobulated, irregular juxtarenal and infrarenal saccular abdominal aortic aneurysm with thickening of the vessel wall and inflammation in the surrounding tissues, consistent with a contained rupture (Figure 1A through 1DFigure 1Computed Tomography and Histopathological and Immunohistochemical Analyses of the Aneurysm.). Pancreatic enzymes and thyroid function were normal. After multiple blood transfusions, the patient underwent a semiemergent open repair of the aneurysm. Intraoperatively, the aneurysm was noted to be intensely inflammatory, and since it had adhered to the adjacent tissues (including the duodenum and visceral vessels), resection and repair were exceedingly difficult.

Histopathological and immunohistochemical analyses of the aneurysmal tissue showed vessel-wall rupture, marked adventitial fibrosis and inflammatory-cell infiltration, occasional eosinophils, obliterative phlebitis, lymphoid follicles, perineural inflammation, and IgG4 plasma-cell infiltration (64% of the overall IgG-positive cells) in both the intima and adventitia, consistent with a ruptured aneurysm associated with IgG4 periaortitis (Figure 1E through 1I). Ten days after aneurysm repair, her serum IgG4 concentration (which had not been measured before surgery, since IgG4 disease was not suspected) was 60.8 mg per deciliter (normal range, 8.0 to 140.0).

IgG4-related diseases have been described primarily in glandular tissues. Since such diseases can be effectively treated with corticosteroids, surgery is seldom necessary.2 Aortic aneurysm arising from isolated IgG4 periaortitis has been previously reported in Japan. It has been suggested that patients with such aneurysms either are asymptomatic or have nonspecific constitutional symptoms and that IgG4-positive cells in such aneurysms are confined to the adventitia. Our findings indicate that this condition also occurs in whites and can present as aneurysmal rupture and that IgG4-positive cells may invade the intima. This condition should be recognized and the role of antiinflammatory therapy (as in glandular IgG4 diseases) in preventing the expansion of the aneurysm and its associated complications should be investigated.

Qi Qian, M.D.
Kianoush B. Kashani, M.D.
Dylan V. Miller, M.D.
Mayo Clinic College of Medicine, Rochester, MN
qian.qi@mayo.edu

2 References

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   Kasashima S, Zen Y, Kawashima A, Endo M, Matsumoto Y, Kasashima F. A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm. J Vasc Surg 2009;49:1264-1271
   CrossRef | Web of Science | Medline

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   Kamisawa T, Okamoto A. IgG4-related sclerosing disease. World J Gastroenterol 2008;14:3948-3955
   CrossRef | Web of Science | Medline

Citing Articles (4)

Citing Articles

1. 1

   Mukul Divatia, Sun A Kim, Jae Y. Ro. (2012) IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease. Yonsei Medical Journal 53:1, 15
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2. 2

   Satomi Kasashima, Yoh Zen. (2011) IgG4-related inflammatory abdominal aortic aneurysm. Current Opinion in Rheumatology 23:1, 18-23
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3. 3

   James R Stone. (2011) Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Current Opinion in Rheumatology 23:1, 88-94
   CrossRef

4. 4

   Wah Cheuk, John K.C. Chan. (2010) IgG4-related Sclerosing Disease. Advances in Anatomic Pathology 17:5, 303-332
   CrossRef