Noninfectious aortitis: what the cardiologist needs to know

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Purpose of review

To sum up a group of noninfectious inflammatory diseases of the aorta and its branches relevant to a cardiologist's daily routine. To describe pathogenetic and clinical advances as well as modern diagnostic tools. To overview most recent treatment options and patient-tailored therapies. To provide an insight in future directions of research.

Recent findings

Pathophysiology of large vessel vasculitides (LVV) are still poorly defined. At least a certain number of patients with idiopathic periaortitis seem to part of the group of IgG4-related diseases which has implications for therapy. Modern diagnostic modalities as Positron-Emission-Tomography (PET)–computed tomography and PET–magnetic resonance tomography proof to be helpful to diagnose or excluded LVV and emerge as long-term surveillance tool. Biological therapy yields varying results but is reported to be important for patients nonresponding or relapsing under glucocorticoid therapy.


Owing to the multifactorial pathogenesis and the small number of cases of LVV further interdisciplinary efforts are necessary to elucidate the pathogenesis of this group of diseases. Technical progress in radiology and nuclear medicine supports clinical, histological, and laboratory findings to increase diagnostic precision. There are several therapies emerging that may have the potential to support patient-tailored treatment approaches in glucocorticoid refractory or relapsing disease.

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