| Abstract [eng] |
Background. Inflammatory abdominal aortic aneurysm (IAAA) is characterized by extensive inflammation of the aortic wall and surrounding tissues, with up to 50% of cases associated with IgG4-related disease (IgG4-RD). A heterogeneous clinical course and a lack of standardized diagnostic guidelines for IgG4-IAAA lead to delayed diagnosis and progressive retroperitoneal fibrosis, resulting in significant organ dysfunction. Aim of the study. To analyze scientific data regarding the etiopathogenesis, diagnosis, and treatment of IgG4-IAAA and to present a clinical case highlighting its diagnostic and therapeutic challenges. Methods. A descriptive literature review and retrospective single-case analysis were performed. PubMed/MEDLINE, Embase, Scopus and Web of Science were searched. According to predefined criteria, 95 peer-reviewed English-language articles were included. Results. Literature analysis revealed that the etiopathogenesis of IgG4-IAAA is dominated by adaptive immunity imbalance, leading to progressive periaortic fibrosis. The pathognomonic "mantle sign" on computed tomography angiography (CTA) is essential for initial suspicion, while inflammatory activity is most accurately differentiated using PET-CT. It was found that up to 50% of patients maintain normal serum IgG4 concentrations; therefore, seronegativity cannot be used as an exclusion criterion. Histopathological examination remains the diagnostic "gold standard" despite biopsy-related risks. Endovascular aneurysm repair (EVAR) is the first-line treatment due to significantly lower perioperative mortality and the avoidance of technically challenging dissection within fibrotic tissues. Open surgical repair remains an alternative in cases of unfavorable EVAR anatomy or the need to mechanically address complications such as ureteral obstruction, gastrointestinal obstruction, or other retroperitoneal organ compression. Medical immunosuppression is crucial for inducing and maintaining remission of active inflammation; however, it is not indicated in the inactive fibrotic stage and may even accelerate aneurysm progression. Conclusions. 1) IgG4-IAAA is an immune-mediated fibroinflammatory pathology characterized by aggressive periaortic fibrosis and asymptomatic, critical aneurysm progression. 2) Diagnosis relies on a multimodal approach (CTA, PET-CT) and histological confirmation due to the low specificity of non-invasive IgG4 serological tests. 3) EVAR is the first-line treatment, while open repair is necessary for unfavorable anatomy or fibrotic complications; immunosuppression is indicated for inducing remission of active inflammation. Keywords. IgG4-related disease; inflammatory abdominal aortic aneurysm; retroperitoneal fibrosis; periaortitis. |
