Multiple Artery Aneurysms: Unusual Presentation of IgG4 Vasculopathy

Abstract

Immunoglobulin G4 (IgG4)-related disease is a chronic systemic disease. It is characterized by inflammatory fibrosis and high serum IgG4 levels. IgG4-positive plasma cells infiltrate target organs in this disease. It may involve the pancreas, biliary tract, lacrimal glands, salivary glands, orbits, thyroid, kidneys, lymph nodes, or retroperitoneum. It may present as vasculitis with involvement of large to medium sized vessels such as the aorta, the common iliac, carotid, and coronary arteries. We present a case of 55-year-old male patient who presented with shortness of breath on exertion and atypical chest pain. On CT angiography, a giant coronary artery aneurysm involving the left anterior descending artery, multiple visceral and intercostal artery aneurysms, and nodular paravertebral soft-tissue thickening secondary to IgG4 vasculopathy.

Keywords: Angiography; Coronary artery aneurysm; Immunoglobulin G4; Vasculitis.

Figure 1:

A 55-year-old male with confirmed IgG4 vasculopathy. Axial maximum intensity projection (MIP) image of CT coronary angiography shows partially thrombosed aneurysm of the left anterior descending artery (arrows).

Figure 2:

A 55-year-old male with confirmed IgG4 vasculopathy. Volume rendered image shows the aneurysm of the left anterior descending artery (in pink).

Figure 3:

A 55-year-old male with confirmed IgG4 vasculopathy. The right anterior oblique caudal view of conventional angiography shows aneurysmal dilatation of the left anterior descending artery with contrast blush (*).

Figure 4:

A 55-year-old male with confirmed IgG4 vasculopathy. Coronal maximum intensity projection images of CT abdominal angiography in arterial (left) and venous (right) phases show partially thrombosed aneurysm in the proximal part of 8^th right intercostal artery (*). Enhancing nodular paravertebral soft-tissue thickening is noted adjacent to the proximal right intercostal arteries above and below this level.

Figure 5:

A 55-year-old male with confirmed IgG4 vasculopathy. Axial image of CT abdominal angiography in the arterial phase shows a partially thrombosed aneurysm in relation to the common hepatic artery. Artifacts are seen in relation to the aneurysm (post-injection in the gastroduodenal artery). The aortic stent graft is also seen in situ.

Figure 6:

A 55-year-old male with confirmed IgG4 vasculopathy. Coronal maximum intensity projection image of CT abdominal angiography shows multiple visceral artery aneurysms – in relation to the common hepatic artery (black arrow), inferior pancreaticoduodenal artery (white curved arrow), and the jejunal branch of superior mesenteric artery (white arrow). Artifacts are noted in relation to the gastroduodenal artery, aorta, and the superior mesenteric artery – representing stent grafts and post-procedural changes. Cholecystectomy clips are also seen.