Immunoglobulin G4-Related Disease Complicating Ruptured Isolated Iliac Artery Aneurysm: A Complex Management Dilemma

Abstract

BACKGROUND Managing IgG4-related disease (IgG4-RD) in the context of vascular complications, such as aneurysms, poses significant challenges, particularly when considering surgical intervention options. The risk of rupture and infection in patients on long-term glucocorticoid therapy complicates treatment decisions. CASE REPORT A 63-year-old woman with a history of IgG4-RD presented with a ruptured right iliac artery aneurysm. She was on long-term oral glucocorticoid therapy. Initial emergency endovascular stent graft implantation was followed by embolization for suspected arterial bleeding and subsequent Salmonella bacteremia. Repeated hospitalizations involved stent graft removal and surgical repair due to persistent infection. Over 2 years, the patient required multiple pelvic drainages and long-term antibiotic and prednisolone therapy, yet her quality of life remained compromised. CONCLUSIONS Our case highlights the unique challenges and considerations in the treatment of IgG4-related aneurysms. Patients with IgG4-RD who are on long-term oral glucocorticoids have an inherent risk of aneurysm rupture. We believe regular follow-ups to monitor the progression of the aorta and iliac arteries into aneurysms are essential. For patients who have developed aneurysms, it is advisable to reduce the dosage of glucocorticoids or even consider surgical treatment as soon as possible. As for the choice of surgical method, there is no consensus yet. While endovascular treatment is less invasive and quicker, it can increase the risk of rupture and bleeding. Open surgery might be a better option. More data are needed to make a definitive judgment.

Figure 1.

Characteristic renal computed tomography (CT) imaging. Low-density lesions on enhanced CT (white arrow).

Figure 2.

(A) Digital subtraction angiography (DSA) intraoperatively before covered stent graft placement showing ruptured bleeding of the common iliac artery aneurysm. (B) Post-implantation imaging after covered stent graft placement. (C) Contrast-enhanced computed tomography angiography (CTA) image after endovascular treatment (white arrows).

Figure 3.

Contrast-enhanced computed tomography angiography (CTA) image after open surgery for the removal of the right iliac artery stent graft (white arrows).