IgG4-related disease may affect many organs, including the cardiovascular system.
Cardiovascular manifestations of IgG4-related disease:
(1) cardiac pseudotumor
(2) inflammatory periaortitis
(3) coronary periarteritis
(4) pericarditis
(5) peripheral periarteritis
The serum IgG4 concentration is significantly elevated.
Various imaging studies can help to localize arterial involvement. 18FDG-PET can identify sites of inflammation.
Histologic examination of an affected site should show a lymphoplasmacytic infiltrate with plasma cells positive for IgG4 on immunostaining.
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Specialty: Immunology/Rheumatology