Small-vessel vasculitis with normal coronary arteries, a possible pattern of IgG4-related disease

Description of Clinical Presentation: A 50-year-old female with a history of documented immunoglobulin G4-related disease (IgG4-RD) and suspected (positive antibodies) systemic lupus erythematosus (SLE) was admitted for a IgG4-RD flare, presenting with fatigue, nausea, and abdominal pain. During hospitalization, the patient developed chest pain and elevated troponin and BNP, with subsequent ECG and coronary angiogram demonstrating no abnormalities. A chest CT with contrast was initially requested to rule out extra-cardiac cause.

Diagnostic Techniques and Their Most Important Findings: Chest CT demonstrated circumferential esophageal wall thickening (Fig 1) and mild wall thickening of the ascending aorta, both findings attributed to IgG4-RD (Fig 2). Additionally, near-circumferential subendocardial hypoattenuation of the left ventricle was seen (Fig 1), raising suspicion for ischemic heart disease. Cardiac MRI was performed for further characterization and exclusion of possible myocarditis.


MRI multiplanar cine SSFP found normal biventricular size and global systolic function, without regional wall motion abnormalities. T1 and T2 mapping images were unreliable due to motion artifacts. Black-blood HASTE images confirmed mild hyperintense ascending aortic wall thickening (Fig 2). Phase-sensitive inversion recovery late gadolinium enhancement (LGE) images showed circumferential diffuse mid-to-apical subendocardial LGE, as well as posteromedial papillary muscle enhancement (Fig 3). LGE was consistent with ischemic disease, and given the normal coronary angiogram and patient’s background, of small-vessel vasculitis.


Follow-up imaging two months after initiation of rituximab and steroids showed resolution of the ascending aorta thickening (Fig 2) and mild improvement of the LGE in the mid ventricle, with persistent circumferential subendocardial LGE in the apical segments and the posteromedial papillary muscle (Fig 1 and 3).

Learning Points from this Case: IgG4-RD is a systemic fibroinflammatory disease, characterized by lymphoplasmacytic infiltrates that can involve nearly any organ. Vasculitis is a well-recognized manifestation of IgG4-RD, which can involve large-sized vessels such as the aorta (1), and in medium-sized vessels, including coronary arteries which typically present with wall thickening and/or pericoronary soft-tissue thickening although coronary stenosis and aneurysm have also been reported (2). While IgG4-RD could theoretically involve all-vessel size (3), to our knowledge, cardiac small-vessel vasculitis is an unknown manifestation of IgG4-RD (1,3), and circumferential ischemic pattern of LGE has not been reported as a manifestation of this condition (4). Importantly, aortitis and diffuse subendocardial LGE are atypical findings for SLE in the absence of coronary artery disease (5-8), and therefore was less favored in our patient with only a suspected SLE based on biomarkers. Our case highlights the possible cardiovascular presentation of IgG4-RD as a small vessel vasculitis with normal coronary arteries, with follow-up imaging showing mild treatment response.

Figure 1. CT and cardiac MRI correlation showing subendocardial hypodensity on CT with contrast (white arrow, A) that correlates with circumferential subendocardial LGE on 4 chamber Turboflash free-breathing PSIR with motion correction (B). Follow-up imaging two months after treatment shows mild improvement of the LGE on Trufi PSIR images (C). Note also esophageal thickening (yellow arrow) on initial CT (A).
Figure 2. CT and cardiac MRI correlation showing aortic wall thickening on initial CT (A) and Haste black-blood (C) with resolution of the aortitis on follow-up CT (B) and MRI Haste black-blood (D) after treatment introduction.
Figure 3. CMR short-axis Turboflash free-breathing PSIR with motion correction shows circumferential subendocardial LGE in the mid (A) to apical (B) left ventricle myocardium and enhancement of the posteromedial papillary muscle (yellow arrow, A), that have mildly improved under treatment in the mid ventricle except for papillary muscle (C) but remains relatively stable at the apex (D).