A challenging case report of IgG4-related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions.

The IgG4-related disease is a distinct, steroid-responsive fibro-inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4-positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri-aortitis, coronary arteritis, and pericarditis. Intra-cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4-related disease with a long-time lag between initiation of symptoms to proper diagnosis with biopsy-proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports.