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Noninfectious aortitis: what the cardiologist needs to know
1Department of Vascular Surgery, Krankenhaus Barmherzige Brüder Regensburg, Regensburg, Germany.
Insights
Large vessel vasculitides (LVV) are inflammatory diseases affecting the aorta. Advances in imaging and emerging therapies offer new hope for patients with difficult-to-treat conditions.
Area of Science:
- Cardiology
- Immunology
- Vascular Medicine
Background:
- Noninfectious inflammatory diseases of the aorta and its branches present diagnostic and therapeutic challenges.
- Pathophysiology of large vessel vasculitides (LVV) remains incompletely understood, with potential links to IgG4-related diseases.
Purpose of the Study:
- To review current knowledge on the pathogenesis, diagnosis, and treatment of LVV relevant to cardiologists.
- To highlight recent advances in diagnostic modalities and therapeutic strategies.
- To explore future research directions in LVV management.
Main Methods:
- Review of recent literature on large vessel vasculitides.
- Analysis of diagnostic advancements, including PET-CT and PET-MR.
- Evaluation of current and emerging treatment options, including biological therapies.
Main Results:
- Positron-Emission-Tomography (PET)-computed tomography and PET-magnetic resonance tomography are valuable tools for diagnosing and monitoring LVV.
- Biological therapies show promise for patients unresponsive or relapsing under glucocorticoid treatment.
- Emerging therapies may facilitate patient-tailored treatment approaches for refractory LVV.
Conclusions:
- Interdisciplinary collaboration is crucial for elucidating LVV pathogenesis due to multifactorial causes and rare occurrences.
- Technological progress in radiology and nuclear medicine enhances diagnostic accuracy.
- New therapeutic avenues are being developed to improve outcomes for patients with glucocorticoid-refractory or relapsing LVV.
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.
Summary:
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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