Background/Purpose:

IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition of uncertain etiology. Reports have described inflammatory aortitis and peri-aortitis in the setting of retroperitoneal fibrosis (RPF) but have not adequately distinguished between these two manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly-defined.  We describe the clinical features, radiology findings, and treatment response in a large cohort of patients with IgG4-RD affecting the large vessels.

Methods:

All patients had biopsy-proven IgG4-RD. Clinical records of all patients diagnosed with IgG4-RD in our Center were reviewed. All radiologic studies were reviewed independently by two vascular radiologists.  We distinguished between primary large blood-vessel inflammation and secondary vascular involvement, defined as disease related to the effects of adjacent inflammation (e.g., peri-aortitis associated with retroperitoneal fibrosis).

Results:

Of the 160 IgG4-RD patients in this cohort, 36 (22.5%) had large-vessel involvement. The mean age of the patients with large-vessel manifestations of IgG4-RD was 54.6 years. Twenty-seven (75%) were male and nine (25%) were female.  Fifteen patients (42%) had primary involvement of their vasculature by IgG4-RD (IgG4-related vasculitis). Inflammatory aortic aneurysms (IAA), which affected eleven patients – 7% of the cohort overall – were observed in the abdominal aorta in seven cases, the thoracic aorta in seven, and both the thoracic and abdominal aorta in three.  IAA of either the thoracic or abdominal aorta or both comprised the most common manifestation of IgG4-related vasculitis.  Three patients had aortic dissection or contained perforation. Peri-aortitis in the setting of RPF accounted for 25 of 27 cases (93%) of secondary vascular involvement by IgG4-RD.  Six patients demonstrated evidence of both primary and secondary blood vessel involvement. Twenty-two of the 36 patients with vascular disease were treated with rituximab and 62.5% showed stabilized or improved radiologic findings following treatment.

Conclusion:

IgG4-RD is a unique, distinctive and treatable cause of large-vessel vasculopathy, comprising both primary vasculitis and secondary vascular involvement. The most common manifestation of IgG4-related vasculitis is inflammatory aortic aneurysm. Peri-aortitis with sparing of the aortic wall is the most common secondary vascular manifestation.

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/large-vessel-involvement-by-igg4-related-disease/