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

Abstract

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.

Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report

“…IgG4-related disease is an autoimmune condition characterized by tissue IgG4 positive plasma cell infiltration [ 4 – 6 ]. Although cardiovascular involvement has been reported, constrictive pericarditis remains an unusual manifestation and often underrecognized presentation [ 6 – 8 ]. No guidelines exist regarding optimal treatment.…”

Multimodality imaging is essential for the diagnosis of multisystem organ involvement of immunoglobulin G4‐related disease

“…We have read with great interest recently published article by Hajsadeghi et al entitled "A challenging case report of IgG4-related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions". 1 We appreciate the authors for describing the IgG4-related disease (IgG4-RD) involving the heart and retroperitoneum in their reports. On the other hand, we consider that there are several drawbacks that need to be addressed.…”

Response to letter to editor regarding a challenging case report of IgG4‐related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions

“…We are grateful for the opportunity to address the points made by Drs Güner et al on our recent case of immunoglobulin G4-related disease (IgG4-RD) involving the heart and retroperitoneum. 1 In response to the mentioned issues, the following points should be highlighted:…”

“…We are grateful for the opportunity to address the points made by Drs Güner et al on our recent case of immunoglobulin G4‐related disease (IgG4‐RD) involving the heart and retroperitoneum. 1 In response to the mentioned issues, the following points should be highlighted: We agree that multimodality imaging is mandatory in any systemic disease, which involves vital organs, and IgG4‐RD is not an exception. So, we planned for 18‐fluorodeoxyglucose positron emission tomography after the diagnosis was confirmed by pathology, seeking for any active site of inflammation, especially the pituitary gland which was postulated to be the first organ to be involved by the disease process.…”