Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice
IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs. It can involve various organs, particularly large vessels. IgG4-related aortitis/periaortitis and periarteriti...
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Frontiers Media S.A.
2025-07-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1625456/full |
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| author | Yan Wang Feng Tian Hui Li |
| author_facet | Yan Wang Feng Tian Hui Li |
| author_sort | Yan Wang |
| collection | DOAJ |
| description | IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs. It can involve various organs, particularly large vessels. IgG4-related aortitis/periaortitis and periarteritis (IgG4-related PAO/PA) predominantly affect the abdominal aorta and iliac arteries, with a higher prevalence in elderly males. This condition exhibits distinct clinical, histologic, and radiological features compared to IgG4-RD without vascular involvement and other immune-associated vasculitides. IgG4-related PAO/PA diagnosis primarily relies on histopathological findings and imaging studies. Glucocorticoids (GCs) are the mainstay of treatment, often combined with immunosuppressants (IMs), while B- and T-cell-targeted therapies are under investigation. Although most patients respond well to treatment, the disease can be life-threatening due to complications such as myocardial infarction, aortic dissection, and aneurysmal rupture. Therefore, understanding these characteristics is crucial for clinicians to make accurate diagnoses and implement effective treatment strategies. This review provides a comprehensive overview of the current understanding of the pathogenesis, histopathological characteristics, clinical features, diagnosis, treatment, and prognosis of IgG4-related PAO/PA. |
| format | Article |
| id | doaj-art-10b0a890d78841fea8db5ea0a67667b4 |
| institution | Kabale University |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Immunology |
| spelling | doaj-art-10b0a890d78841fea8db5ea0a67667b42025-08-20T03:29:11ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-07-011610.3389/fimmu.2025.16254561625456Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practiceYan WangFeng TianHui LiIgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs. It can involve various organs, particularly large vessels. IgG4-related aortitis/periaortitis and periarteritis (IgG4-related PAO/PA) predominantly affect the abdominal aorta and iliac arteries, with a higher prevalence in elderly males. This condition exhibits distinct clinical, histologic, and radiological features compared to IgG4-RD without vascular involvement and other immune-associated vasculitides. IgG4-related PAO/PA diagnosis primarily relies on histopathological findings and imaging studies. Glucocorticoids (GCs) are the mainstay of treatment, often combined with immunosuppressants (IMs), while B- and T-cell-targeted therapies are under investigation. Although most patients respond well to treatment, the disease can be life-threatening due to complications such as myocardial infarction, aortic dissection, and aneurysmal rupture. Therefore, understanding these characteristics is crucial for clinicians to make accurate diagnoses and implement effective treatment strategies. This review provides a comprehensive overview of the current understanding of the pathogenesis, histopathological characteristics, clinical features, diagnosis, treatment, and prognosis of IgG4-related PAO/PA.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1625456/fullIgG4-related diseasevascular involvementretroperitoneal fibrosisdiagnostic criteriatreatment strategies |
| spellingShingle | Yan Wang Feng Tian Hui Li Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice Frontiers in Immunology IgG4-related disease vascular involvement retroperitoneal fibrosis diagnostic criteria treatment strategies |
| title | Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice |
| title_full | Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice |
| title_fullStr | Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice |
| title_full_unstemmed | Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice |
| title_short | Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice |
| title_sort | unraveling the complexity of igg4 related aortitis and periarteritis from pathogenesis to clinical practice |
| topic | IgG4-related disease vascular involvement retroperitoneal fibrosis diagnostic criteria treatment strategies |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1625456/full |
| work_keys_str_mv | AT yanwang unravelingthecomplexityofigg4relatedaortitisandperiarteritisfrompathogenesistoclinicalpractice AT fengtian unravelingthecomplexityofigg4relatedaortitisandperiarteritisfrompathogenesistoclinicalpractice AT huili unravelingthecomplexityofigg4relatedaortitisandperiarteritisfrompathogenesistoclinicalpractice |