ArticleArthritis research & therapy2017
Clinical features of IgG4-related periaortitis/periarteritis based on the analysis of 179 patients with IgG4-related disease: a case-control study.
Article in Arthritis research & therapy, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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35 citing papers in PubMed, 100 citations in OpenAlex.
- Abdominal aortic aneurysm as an IgG4-related disease.Clinical and experimental immunology · 2019Trial
- Inflammatory Aortopathies in Rheumatic Diseases: A State-of-the-Art Review.Diagnostics (Basel, Switzerland) · 2026Review
- Management of IgG4-Related Disease.Current rheumatology reports · 2026Review
- A rare case of immunoglobulin G4-related periarteritis mimicking pancreatic cancer.Clinical journal of gastroenterology · 2026Article
- Article
- Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice.Frontiers in immunology · 2025Review
- Common Femoral Artery Aneurysm: A Rare Manifestation of Immunoglobulin G4-Related Disease.Annals of vascular diseases · 2025Article
- Three cases of IgG4-RD complicated by splenic artery aneurysm: a complication of IgG4-RD?RMD open · 2024Article
- Steroid-resistant immunoglobulin G4-related coronary arteritis: a case report.European heart journal. Case reports · 2024Article
- Coronary Artery Aneurysm or Ectasia as a Form of Coronary Artery Remodeling: Etiology, Pathogenesis, Diagnostics, Complications, and Treatment.Biomedicines · 2024Review
- Multimodality Imaging Features of Immunoglobulin G4-related Vessel Involvement.Radiology. Cardiothoracic imaging · 2024Article
- Immunoglobulin G4-Related Disease Complicating Ruptured Isolated Iliac Artery Aneurysm: A Complex Management Dilemma.The American journal of case reports · 2024Article
- Association of autoimmune pancreatitis with Raghib syndrome.Clinical case reports · 2023Article
- Thoracic Aorta: Anatomy and Pathology.Diagnostics (Basel, Switzerland) · 2023Review
- Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes - preliminary data.Renal failure · 2022Article
- Multimodality Imaging Evaluation of Coronary IgG4-Related Disease: A "Tumor-Like" Cardiac Lesion.Diagnostics (Basel, Switzerland) · 2022Article
- Spectrum of Large and Medium Vessel Vasculitis in Adults: Primary Vasculitides, Arthritides, Connective Tissue, and Fibroinflammatory Diseases.Current rheumatology reports · 2022Review
- Amendment of the Japanese consensus guidelines for autoimmune pancreatitis, 2020.Journal of gastroenterology · 2022Review
- Decisive diagnostic clue for infectious abdominal aortic aneurysm caused byFrontiers in cardiovascular medicine · 2022Article
- Significance of high serum IgG4 in complete or non-full-fledged IgG4-related disease-a retrospective investigation of 845 patients and its clinical relevance.Clinical rheumatology · 2022Article
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Authors and funding
9 authors at 3 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundImmunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic condition characterized by high serum immunoglobulin G4 (IgG4) concentration and IgG4-bearing plasma cell infiltration in affected organs. Although it has become evident that IgG4-RD also involves the systemic aortic/arterial system, the precise details of this condition remain unclear. The present study sought to clarify the clinical features of IgG4-related periaortitis/periarteritis.
methodsAmong 223 patients with IgG4-RD, 179 (131 male, median onset age 67 years) were recruited for this study. Periaortitis/periarteritis was defined as vessel wall thickness with circumferential enhancement on contrast-enhanced computed tomography.
resultsPeriaortitis/periarteritis was identified in 65 (36.3%; 53 male) of 179 IgG-RD patients. The distribution of IgG4-related periaortitis/periarteritis could be broadly classified into five types, with the most prevalent Type 2 (44.6%) being localized at the infra-renal artery portion of the abdominal aorta and continuing to the iliac arteries. The infra-renal artery region of the abdominal aorta was most frequently involved (>80%) among IgG4-related periaortitis/periarteritis cases. Comparisons of clinical parameters between IgG4-RD patients with and without periaortitis/periarteritis revealed significantly higher propensities for older IgG4-RD onset age and highly active disease state featuring elevated serum IgG, IgG4, circulating immune complex, and soluble interleukin-2 receptor. All patients showed improvement of wall thickening after steroid therapy, although nine patients (20.9%) exhibited worsening of luminal dilatation. The main risk factor for this manifestation was prior luminal dilatation according to multivariate analysis.
conclusionIgG4-related periaortitis/periarteritis predominantly occurred at the infra-renal artery portion of the abdominal aorta, affected older IgG4-RD onset patients, and was prevalent in highly active disease states. As reported previously, the main risk factor for worsening luminal dilation after corticosteroid therapy was the existence of luminal dilation beforehand.
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