Description

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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