Non-infectious aortitis of the ascending aorta: a histological and clinical correlation of 71 cases including overlap with medial degeneration and atheroma—a challenge for the pathologist

Ryan, Carmel B.; Barbour, A. D.; Burke, Louise M.; Sheppard, Mary N.

doi:10.1136/jclinpath-2015-203061

Cited by 23 publications

(6 citation statements)

References 20 publications

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“…At present there is a small but important body of literature available regarding histopathologically proven aortitis. Although multiple surgical series have identified idiopathic aortitis as an incidental histopathologic finding after aortic root/arch surgery , clinical outcomes in these patients have been difficult to compare due to considerable heterogeneity in definitions of clinical phenotypes, approaches to treatment, monitoring, and duration of follow‐up.…”

Section: Discussionmentioning

confidence: 99%

Outcomes Among 196 Patients With Noninfectious Proximal Aortitis

Clifford

Arafat

Idrees

et al. 2019

Arthritis & Rheumatology

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Objective. Noninfectious aortitis may occur in the context of a recognized systemic disease or as a topographically limited lesion without systemic features, which is called clinically isolated aortitis (CIA). This study was undertaken to better define and stress the limitations of this diagnostic category in a large population of patients in a single center dedicated to aortic diseases and to suggest recommendations for care.Methods. Records of patients undergoing thoracic aortic surgery (1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012) at the Cleveland Clinic were reviewed to identify 196 patients with histopathologically proven aortitis. Clinical diagnoses (giant cell arteritis [GCA], Takayasu arteritis [TAK], CIA, or Other) were determined at the time of surgery. Clinical features, laboratory findings, and imaging results were recorded throughout the follow-up period. At least 6 months of follow-up data were available for 73 CIA patients.Results. The mean age of the patients at time of surgery was 65.6 years (range 15-88 years); 67% of patients were female, and 90.3% were white. At the time of surgery, 129 patients (65.8%) met criteria for CIA, 42 (21.4%) for GCA, 14 (7.1%) for TAK, and 11 (5.6%) met criteria for other systemic inflammatory diseases. During a mean followup period of 56.2 months, 19% of CIA patients developed new symptoms, 45% developed new radiographic vascular lesions, 40% underwent additional vascular surgery, and 12% died (n = 9). Eleven of 73 patients (15%) initially classified as having CIA developed features of a systemic disease, most often GCA.Conclusion. The majority of patients (66%) with histopathologically proven aortitis have CIA at the time of surgery. CIA patients infrequently report new symptoms over time, but new vascular lesions requiring surgery commonly occur. Serial follow-up including large vessel imaging is strongly advised for all aortitis patients.

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Section: Discussionmentioning

confidence: 99%

Outcomes Among 196 Patients With Noninfectious Proximal Aortitis

Clifford

Arafat

Idrees

et al. 2019

Arthritis & Rheumatology

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“…In our opinion, effort should be made to reach a global classification for all LVVs. Thus, conditions with potential overlap with TAK and GCA, such as isolated aortitis and giant cell aortitis [90,91], should be incorporated into a new classification wherever possible. Accurate phenotyping of LVV subtypes is required in order to obtain homogeneous groups for research studies and clinical trials, and ultimately for optimal clinical care [92].…”

Section: Differentiation Of Tak From Other Large Vessel Vasculitidesmentioning

confidence: 99%

Takayasu arteritis: advanced understanding is leading to new horizons

2018

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Although outcomes in Takayasu arteritis (TAK) are improving, diagnosis is typically delayed and significant arterial injury accrues. While wider use of non-invasive imaging is impacting this, the onus remains with clinicians to consider a diagnosis of TAK earlier. Meanwhile, morbidity and mortality in TAK remains increased. Herein we review the current situation, outline recent advances and summarize remaining challenges. Understanding of disease pathogenesis remains poor. However, recent genetic data and identification of pathogenic cytokines may facilitate the search for biomarkers capable of distinguishing active and inactive disease, inflammatory and non-inflammatory arterial remodelling. Imaging is critical for TAK, and each modality has important strengths and limitations. Dependence upon CS therapy remains too high. However, the impact of combination immunosuppressive therapy is now recognized, biologic therapies are increasingly available and new agents offer promise. Multicentre clinical trials are now required, and these will depend upon development of defined clinical and imaging end-points.

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“…ied histologically, aortitis associated with autoimmune diseases is at least as frequent as GCA and TAK (13). The reason why clinicians underestimate involvement of the aorta in autoimmune diseases is probably that clinical and laboratory signs of inflammation are easily attributed to the underlying primary condition and that vascular imaging techniques are not routinely used in this group of patients.…”

Section: Editorialmentioning

confidence: 99%