Key result
Corticosteroid therapy is associated with markedly improved survival in idiopathic aortitis.
Why the study?
Because idiopathic aortitis is rare, data on its diagnosis and treatment are limited and derived mainly from small observational studies, motivating a comprehensive synthesis of available evidence.
Do specific management strategies such as corticosteroid administration and clinical surveillance improve survival in patients with idiopathic aortitis?
Meta-Analysis (n=79)
Do specific management strategies such as corticosteroid administration and clinical surveillance improve survival in patients with idiopathic aortitis?
This systematic review and meta-analysis of 79 idiopathic aortitis cases demonstrates a high overall mortality rate of 20%—particularly in younger patients and those with descending aorta involvement—while showing that corticosteroid therapy and longitudinal clinical surveillance are strongly associated with survival.
Answers are grounded in this paper and cite their sources.
May support corticosteroids and surveillance in idiopathic aortitis; leaves open prospective confirmation before practice change.
Introduction Idiopathic Aortitis (IA) is defined as inflammation of the aortic wall, in the absence of a systemic disease or an infection. Most cases are found incidentally during repair of thoracic aortic aneurysms. Given the rarity of the disease, data on IA diagnosis and treatment are limited and derived mainly from small observational studies. This study aims to comprehensive and up-to-date synthesis of the available evidence on IA. Methods Literature search of the PubMed, Scopus, and Web of Science databases was performed in late September 2025. Data regarding patients’ medical history, presentations, complications, diagnostic investigations, management and outcomes were obtained. Results A total of 58 studies were included, comprising data from 79 patients with idiopathic aortitis. The mean age was 46 ± 20 years, with a slight male predominance of 53%. Weight loss was the most frequently reported symptom (58%). Regarding management, 44% underwent exclusive surgical treatment, 31% received mixed therapy, and 25% were managed medically. Corticosteroids were utilized in 51% of cases. The overall mortality rate was 20%. Significant predictors of mortality included a younger age at presentation (18 vs. 48 years, P < 0.001) and descending aorta involvement ( P = 0.005). Conversely, corticosteroid administration ( P = 0.002) and clinical surveillance ( P < 0.001) were significantly associated with survival. Conclusion This meta-analysis highlights idiopathic aortitis as a severe condition with significant mortality whereas the administration of corticosteroids and clinical surveillance are strongly associated with survival, our findings emphasize the need for standardized, evidence-based management protocols and prospective multicenter studies to optimize patient outcomes.
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Hamdan et al. (2026) conducted a meta-analysis in Idiopathic aortitis (n=79). Corticosteroids vs. No corticosteroids was evaluated on Corticosteroid utilization in patients who survived vs. died (mortality status comparison) (p=0.002). Corticosteroid administration (63% in survivors vs. 0% in deceased, P = 0.002) and clinical surveillance (81% vs. 0%, P < 0.001) were significantly associated with survival in idiopathic aortitis.
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