Imaging findings in cardiovascular involvements of IgG4-related disease: a systematic review study

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by IgG4-positive plasma cell infiltration that can affect multiple organs, including the cardiovascular system. The diagnosis of IgG4-RD relies on a combination of clinical, serological, radiological and pathological findings. However, due to the varied and insidious clinical presentations, normal IgG4 levels in a significant percentage of patients and frequent multi-organ involvement, imaging plays a crucial role in the diagnosis of IgG4-RD. The aim of this study is to comprehensively examine the imaging findings in IgG4-related cardiovascular disease for accurate diagnosis and appropriate treatment.

A systematic search was conducted across the electronic databases PubMed, Scopus, Embase and Web of Science, to 1 September 2023, following PRISMA guidelines, searching for studies reporting detailed cardiovascular imaging findings in IgG4-RD.

The search yielded 68 studies (60 case reports, 5 case series, 2 cross-sectional, 1 case-control) with 120 cases of cardiovascular IgG4-RD. Most of the cases were male, averaging 62.8 years. The common initial symptoms were dyspnoea and chest pain. The most common imaging finding was vasculopathy, including vessel wall thickening, periarteritits, periaortitis, aortitis, stenosis, ectasia, aneurysm formation, intramural haemorrhage, fistula formation and dissection, followed by pericardial involvement and mediastinal masses. Case series and cross-sectional studies also showed vasculopathy to be the most common finding on various imaging modalities, including angiography and PET/CT, highlighting the complex pathology of IgG4-RD.

This study evaluated current IgG4-RD articles, revealing a higher prevalence in men and vasculopathy as the most common cardiovascular complication.