Background/Purpose:

Idiopathic aortitis (IA) is a poorly defined entity with no specific pathological or clinical criteria for its classification or diagnosis, except for the presence of aortic inflammation and absence of clinical features of another systemic condition. Most of the present knowledge about IA comes from retrospective series of cases with pathologically identified aortitis. The objective of this systematic review was to clarify available data regarding outcomes in patients treated with corticosteroids and those without treatment.

Methods:

Data sources: Search strategy using terms “aortitis” and “idiopathic/isolated” identified published articles and abstracts using MEDLINE and EMBASE databases.

Study selection: Inclusion criteria required that cases of idiopathic aortitis be confirmed by pathology. Abstracts and case reports were excluded except for abstracts that complemented published manuscripts; reviews were used for triangulation. Authors were contacted for clarifications when required.

Data extraction: Data were extracted independently by two reviewers. Our primary goal was to compare outcomes between patients treated with corticosteroids and untreated patients. The primary outcomes included: development of new vascular lesions, clinical complications, delayed diagnosis of other rheumatological diseases and vascular death. Secondary outcomes included preoperative symptoms, ESR/CRP levels and presence of branch vessel disease.

Results:

Search yielded 262 records of which 10 fulfilled inclusion criteria. The majority of studies were retrospective. Even the few studies that reported on treatment status described very few events of interest: Miller et al described no new vascular lesions in two treated patients versus one lesion in 19 untreated patients while Clifford et al reported 2 new vascular lesions in 11 treated and 27 in 54 untreated patients. Wang et al reported one new vascular lesion (new aortic aneurysm) in the treated group and none in the untreated patients. Two studies described the need for re-operation, reported in two treated and none of the untreated patients in each of the studies. Finally, Liang et al reported 4 deaths (of unspecified cause) among untreated patients and none in the treated group.

Conclusion:

Few studies report clinical outcomes in patients diagnosed with idiopathic aortitis treated with corticosteroids. Consequently, there exists a lack of data to guide therapy and long term management of these individuals. The retrospective nature and small cohorts from which data is extracted make it difficult to draw conclusions. Future studies are required to better understand outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/corticosteroid-use-in-idiopathic-aortitis-a-systematic-review/