Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by multiorgan lymphoplasmacytic infiltration, obliterative
phlebitis and storiform
fibrosis. It can be associated with cardiovascular pathology. The objective of this narrative review is to summarise the published literature on cardiovascular manifestations of
IgG4-RD and to provide a basis for diagnosis and management of the condition by the practising cardiologist.We propose the following categorisations of cardiovascular
IgG4-RD:
aortitis, medium-vessel
arteritis, pulmonary
vascular disease,
phlebitis, valvulopathy,
pericarditis,
myocardial disease and antineutrophilic cytoplasmic antibody-associated
vasculitis. We also review herein developments in radiological diagnosis and reported medical and surgical
therapies. Cardiovascular lesions frequently require procedural and/or surgical interventions, such as
aortic aneurysm repair and valve replacement.
IgG4-RD of the cardiovascular system results in serious complications that can be missed if not evaluated aggressively. These are likely underdiagnosed, as clinical presentations frequently mimic
cardiovascular disease due to more common aetiologies (
myocardial infarction,
abdominal aortic aneurysm and so on). While systemic
corticosteroids are the mainstay of
IgG4-RD treatment,
biological and disease-modifying agents are becoming more widely used. Cardiologists should be aware of cardiovascular
IgG4-RD as a differential diagnosis, and understand the roles of
corticosteroids, disease-modifying agents and
biologicals, as well as their integration with surgical approaches. There are several knowledge gaps, including diagnosis, risk factors, pathogenesis and appropriate management in Ig4-RD of the cardiovascular system. Areas lacking well-conducted randomized trials include safety of
steroids in the setting of vascular
aneurysms and the role of disease-modifying drugs and
biological agents in patients with established cardiovascular complications of this multifaceted enigmatic disease.