IgG4-related Disease Involving the Cardiovascular System: An Intracardiac Mass and a Mass Lesion Surrounding a Coronary Artery

Abstract

A 61-year-old Japanese man with IgG4-related autoimmune pancreatitis developed a mass in the right atrium (RA) and a mass lesion surrounding the left anterior descending coronary artery. We performed an intracardiac echo catheter-guided percutaneous biopsy of the RA mass, and histologically diagnosed it as IgG4related disease. Oral corticosteroid therapy gradually downsized the mass lesions. We encountered a very rare case with mass lesions in the cardiovascular system of the IgG4-related disease that were able to be diagnosed using an intracardiac echo-guided biopsy.

Immunoglobulin G4-related masses surrounding coronary arteries: a case report

“…IVUS was performed in several previous reports on IgG4-related coronary arteritis and demonstrated only thickening of the adventitia. 10 , 11 , 15 In contrast, IVUS images of coronary arteries in our case showed the thickening of the adventitia and multiple microchannels, which were thought to be the feeding arteries to the masses, originally detected by coronary angiography, originating from the affected coronary arteries. The images of coronary angiography and IVUS in our case suggested that the feeding arteries from the affected coronary arteries may contribute to the mass formation in IgG4-related coronary arteritis.…”

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

“…We performed a literature review using the keywords “Immunoglobulin G4‐Related Disease” and “intracardiac/ cardiac mass” or “cardiac pseudotumor” in PubMed, Embase, and Scopus databases to search for relevant articles. Table 1 summarizes the 10 identified cases of IgG4‐RD with intra‐cardiac mass including the present case 10–18 . The mean age of all cases was 49.5 years (range: 27–69).…”

“…In eight of nine of the published cases, echocardiography was one of the imaging modalities used for visualizing the cardiac mass. In six cases, the right heart was involved with an infiltrating mass in three different locations: the right ventricular apex, the right atrium with extension to the inter‐atrial septum and/or superior vena cava, and the right ventricular outflow tract 11,13–15,17,18 . The most common structure involved by the mass was the RA.…”

Multisystem IgG4-related disease involving the abdomen and coronary arteries and causing chronic abdominal pain

“…IgG4RD was originally discovered in patients with autoimmune pancreatitis accompanied by elevated serum IgG4 levels 1,2 and it has been described in almost every organ system including the pancreas, bile ducts, lacrimal glands, salivary glands, central nervous system, thyroid, lung, liver, gastrointestinal tract, kidney, prostate, retroperitoneum, cardiovascular system, lymph nodes, skin, and breast. [1][2][3][4] IgG4-RD affecting the heart is relatively rare, with most reported cases involving the coronary arteries followed by pericardial, aortic, myocardial, and valvular lesions. 4,[5][6][7][8][9] The disease entity is characterized by diffuse lymphoplasmacytic infiltrates and the presence of abundant IgG4positive plasma cells in extensive fibrosis, frequently associated with tumorous swelling lesions and elevated serum IgG4 concentrations.…”

“…IgG4-related disease (IgG4RD) was originally discovered in patients with autoimmune pancreatitis accompanied by elevated serum IgG4 levels 1,2 and has subsequently been described in almost every organ system. [1][2][3][4] IgG4related cardiovascular disease can affect the coronary arteries, heart valves, myocardium, pericardium, aorta, and peripheral vasculature. 4 IgG4RD is characterized by diffuse lymphoplasmacytic infiltrates and the presence of abundant IgG4-positive plasma cells within extensive fibrosis, frequently associated with tumorous swelling and elevated serum IgG4 concentrations.…”