Multiorgan IgG4-related vasculitis: dissecting abdominal aortic aneurysm, renal pseudotumor and fulminant bilateral visual loss: a systematic review
DOI:
https://doi.org/10.70577/1gfsa660Keywords:
IgG4-related disease, vasculitis, dissecting aortic aneurysm, pseudotumor, visual loss, aortitis, multiorgan diseaseAbstract
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.
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Copyright (c) 2026 Diego Roberto Orrala Mendoza, Ivana Elizabeth Flores Ramírez, Luis Felipe Oviedo Vásquez, Fernando David Rivera Pérez, Arleth Bianka Sánchez Pacheco (Autor/a)
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