Dataset Information

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

ABSTRACT:

Background

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with high serum IgG4 levels affecting various organs, such as the pancreas, lacrimal and salivary glands, thyroid, kidney, and lung. Typical cardiovascular manifestations of IgG4-RD include periaortitis, coronary arteritis, and pericarditis. However, reports of IgG4-RD associated with coronary arteritis are rare. Here, we report a case of IgG4-related masses surrounding the coronary arteries.

Case summary

A 59-year-old man was referred to our hospital because of mediastinal masses detected by computed tomography (CT). Coronary CT angiography revealed masses surrounding the right coronary artery and the left anterior descending coronary artery. An elevated serum level of IgG4 and histological findings led to the diagnosis of IgG4-related coronary arteritis with mass formation. Coronary angiography showed numerous feeding arteries to the masses, which were demonstrated as multiple microchannels in the intravascular ultrasound (IVUS) images.

Discussion

IgG4-RD involving the cardiovascular system has been reported. However, coronary artery disease associated with IgG4-RD is very rare, and the mechanism of mass formation in IgG4-related coronary arteritis is unclear. In our case, within the cardiovascular system, IgG4-RD was limited to the coronary arteries, suggesting that the affected coronary arteries may provide the necessary blood supply to the mass, thus, aiding its growth. These findings were supported by the images from coronary angiography and IVUS.

SUBMITTER: Nakamura T

PROVIDER: S-EPMC8186918 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Immunoglobulin G4-related disease (IgG4-RD) has recently been recognized as a systemic fibroinflammatory disease characterized by IgG4-positive lymphoplasmacytic infiltration. Despite the increased awareness of this disease category, cardiovascular involvement has rarely been reported. An 83-year-old man was admitted because of a moderate amount of pericardial effusion concomitant with bilateral pleural effusion. Despite aggressive heart failure therapy, pericardial effusion increased. He underwent pericardial drainage, and heart failure symptoms significantly improved. After only 1 month, right-sided heart failure with re-accumulation of pericardial fluid recurred, although the results of cardiac catheter examination were not indicative of constrictive pericarditis. Despite the aggressive medication, he developed weight gain and pleural effusion 6 months after pericardial drainage. Based on multimodal hemodynamic and morphological evaluations, he was finally diagnosed with effusive constrictive pericarditis and underwent total pericardiectomy. Based on histopathological findings, the final diagnosis was established as IgG4-RD associated constrictive pericarditis. We report an extremely rare case of IgG4-RD associated pericardial involvement, in which rapid transition from cardiac tamponade to effusive constrictive pericarditis was confirmed. Diagnostic work-up for constrictive pericarditis requires a high index of suspicion. Increased recognition of IgG4-RD associated pericardial involvement could potentially mitigate morbidity and might contribute to improved patient outcomes. <Learning objective: Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by chronic IgG4-positive lymphocyte infiltration that causes fibrosclerotic changes in various tissues and organs. Detailed serial multimodal imaging and physical examination contribute to the recognition of latent constrictive physiology and clinical decision-making. Increased recognition of IgG4-RD associated pericardial involvement could lead to early diagnosis and might contribute to improved patient outcomes.>.