Vasculitis por IgG4 multiorgánica: aneurisma disecante de la aorta abdominal, pseudo-tumor renal y pérdida visual bilateral fulminante: revisión sistemática
Year: 2026
Type: article
Study design: Systematic Review
Abstract: Introduction: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterized by infiltration of IgG4-positive plasma cells in multiple organs, which can affect the cardiovascular system with life-threatening complications such as aneurysms and aortic dissection (Zen et al., 2012; Ghaffari Jolfayi et al., 2025). Multiorgan involvement, including renal pseudotumors and orbital involvement, can present synchronously or metachronously, posing a diagnostic challenge (Maccagno et al., 2022; Haraguchi et al., 2012). Objective: To synthesize the available evidence on multiorgan IgG4-related vasculitis with manifestations of dissecting abdominal aortic aneurysm, renal pseudotumor and fulminant bilateral visual loss. Methodology: Systematic review following PRISMA 2020 guidelines (Page et al., 2021). A systematic search was conducted in PubMed, LILACS, SciELO and Cochrane for studies published between 2000 and 2026 on IgG4-related vasculitis with aortic involvement, pseudotumors and ophthalmological manifestations. Results: Eight relevant studies documenting the association between IgG4-RD and cardiovascular, renal and orbital manifestations were identified. IgG4-related aortitis/periaortitis predominantly affects the abdominal aorta and iliac arteries, with higher prevalence in elderly males (Ghaffari Jolfayi et al., 2025; Zen et al., 2012). IgG4-related pseudotumors are extremely rare lesions, representing approximately 0.05% of evaluated lesions (Maccagno et al., 2022). Orbital IgG4 involvement can cause compressive optic neuropathy with fulminant visual loss, which responds favorably to systemic corticosteroids (Haraguchi et al., 2012). Conclusion: Multiorgan IgG4-related vasculitis is an exceptional but potentially lethal entity that requires a high index of suspicion. Synchronous presentation of aortic aneurysm, renal pseudotumor and bilateral visual loss should suggest the possibility of IgG4-RD, and early diagnosis with imaging studies and serum IgG4 measurement is essential to initiate timely immunosuppressive therapy and reduce associated morbidity and mortality.
DOI: 10.70577/1gfsa660
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W7207613271