Vaskulitis der Koronararterien - American College of Cardiology (2023)

Vasculitis is a general term for inflammation of the blood vessel walls, which can lead to stenosis, occlusion, aneurysm, or rupture. While the definition itself may seem simple at first, the disorders that fall into this category are difficult to diagnose and treat due to their rarity, complexity, vascular distribution, and multiple organ involvement. A thorough understanding of the possible etiologies is necessary since vasculitis can be primary or secondary to another autoimmune disease or associated with other triggers such as drugs, infections or malignancies.1-3Cardiac manifestations in primarily systemic vasculitis occur with varying frequency and can affect all layers of the heart.4In a patient with vasculitis, cardiac involvement indicates a poor prognosis.5Therefore, understanding when to consider vasculitis in patients with coronary artery abnormalities, how to initiate an investigation, and to whom it should be referred for further investigation is crucial to the efficient and effective management of these patients.

The prevalence of coronary arteritis in patients with systemic vasculitis is largely unknown due to the relative rarity of these diseases and the lack of standardized prospective imaging studies to systematically evaluate the coronary vasculature. In addition, involvement of the subepicardial coronary microcirculation can lead to symptoms of myocarditis or cardiomyopathy, but these blood vessels are missed by conventional coronary angiography and noninvasive cardiac imaging.6In addition, coronary arteritis may be asymptomatic in some patients, and in others clinically significant coronary artery disease may be secondary to accelerated atherosclerosis rather than active inflammation of the artery wall.7Therefore, not all coronary artery disease in patients with vasculitis is due to active and uncontrolled inflammation of the coronary arteries.

The clinical, laboratory, and physical examination characteristics of primary systemic vasculitis are very different and can overlap significantly. The most common findings associated with some of these disorders are listed in Table 1. Isolated coronary arteritis without other systemic symptoms is considered extremely rare.8However, patients with vasculitis may have nonspecific symptoms attributed to other etiologies or may remain unrecognized for several months or years prior to diagnosis; Therefore, a high index of suspicion is required in providers of primary care, cardiology and vascular medicine.

Table 1: Diseases highlighted by coronary arteritis or periarteritis

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Tabla 1

Vaskulitis der Koronararterien - American College of Cardiology (2)

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Clinical features that should indicate the possibility of underlying vasculitis in patients with coronary artery symptoms include: unexplained elevated inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) or constitutional symptoms (fever, chills, night sweats, weight loss); asymmetric or absent radial pulses; subclavian or aortic murmurs; multifocal visceral infarcts without embolic etiology; clinically significant coronary artery stenosis in patients ≤ 40 years or in patients without cardiac risk factors. However, since patients with coronary arteritis may present with acute symptoms of cardiac ischemia, it may not be possible in these circumstances to investigate symptoms suggestive of systemic vasculitis before proceeding with emerging coronary artery reperfusion.

Although there are no specific features on conventional coronary angiography that diagnose coronary arteritis, certain features can suggest it. For example, a smooth conical narrowing has been described in giant cell arteritis (GCA) with coronary involvement.9In Takayasu's arteritis, three main types of coronary lesions have been described based on angiographic and histological analyses: Type 1, stenosis or occlusion of ostium or proximal segments; Type 2, diffuse or focal coronary arteritis, which may diffusely spread to all epicardial branches or involve focal segments (so-called crack lesions); and type 3, coronary aneurysms.10In patients with coronary lesions, type 1 is most common (60-80%), while type 2 is less common (10-20%) and type 3 is considered rare (0-5%).10-12Large aneurysms should raise suspicion of previous damage to the epicardial coronary arteries from Kawasaki disease that has not resolved, or indicate the possibility of polyarteritis nodosa (PAN).13The latter should be considered particularly in multifocal aneurysms with a bead-on-a-string or nodular appearance.5Any combination of the above findings may occur in patients with vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) and Bechet's disease.14

Although lumen assessment with conventional coronary angiography helps to understand coronary anatomical abnormalities, it is insufficient to assess inflammation or thickening of the coronary artery wall or to identify extracoronary arterial abnormalities. Because isolated coronary arteritis rarely occurs without other arterial or systemic findings, computed tomography (CT) or magnetic resonance angiography (MRI) should be considered in patients with suspected or diagnosed coronary arteritis. In patients with large (GCA and Takayasu's arteritis), moderate (PAN), or variable (Behcet's disease) vasculitis, evaluation with noninvasive angiography of the chest, abdomen, and pelvis is strongly recommended. In patients with suspected small vessel vasculitis (ie, related to ANCA), a chest CT scan with and without contrast to evaluate changes in the lung parenchyma and cardiac MRI to evaluate non-coronary abnormalities (pericarditis) are recommended. . . . The above modalities are also beneficial in the assessment of disorders leading to narrowing of the lumen due to periarterial soft tissue thickening or extrinsic compression, as seen in immunoglobulin G4 (IgG4)-related disorders or less common disorders such as Erdheim's Chester disease is the case.15-16Positron emission tomography (PET) can help assess hypermetabolism in the great vessels (aorta and first-order branches), which can be helpful in diagnosing GCA and Takayasu's arteritis, but is less useful for detecting inflammation in to assess the coronary arteries and low sensitivity for medium or small vascular vasculitis. Nuclear medicine myocardial perfusion studies can help determine myocardial viability but cannot distinguish between reduced perfusion and vasculitic or atherosclerotic etiology.17However, because such perfusion studies are less useful in diagnosing coronary arteritis, they can provide useful information to determine whether CABG would be beneficial and, if so, what endpoints should be considered.

Data on interventions in patients with coronary arteritis, regardless of etiology, are limited to case reports and retrospective cohort studies. To date, no prospective randomized study has been conducted to determine the optimal timing or method of revascularization. Although the sample size is limited, the most complete outcome data was generated in patients with Takayasu's arteritis. A recent meta-analysis comparing the type of revascularization based on the location of the lesion found that restenosis/coronary insufficiency was more common with the endovascular intervention than with the open surgical approach (odds ratio 7.38, 95% CI 2.36- 23, 10). , p=0.01).18Although conventional CABG, angioplasty, and transaortic coronary ostium endarterectomy have been performed with reasonable results in patients with Takayasu's arteritis, no study has demonstrated a definitive advantage of one approach over the other. Therefore, decisions about approach must be tailored to the patient based on the location(s) and extent of the coronary artery lesion(s). In addition, the distribution and severity of the inflammation of the aortic vessels and arch of the aorta must be considered when determining the appropriate site for the proximal graft anastomosis. Particular attention should also be paid to the choice of graft site in patients with Takayasu's arteritis, since internal mammary artery grafts may need to be avoided if the subclavian artery or brachiocephalic trunk are involved.19In general, observational studies suggest that surgery should be avoided during the active stage of inflammation to increase the chances of success.20In circumstances where patients are not considered candidates for surgery and percutaneous coronary intervention is deemed necessary, limited data suggest that drug-eluting stents may have a reduced risk of restenosis compared to bare stents.21Irrespective of the endovascular or surgical approach, continuous drug treatment of the underlying vasculitis with immunosuppressive therapy is essential.

Individual treatment regimens for systemic vasculitis are beyond the scope of this review, but general considerations are presented in Table 1. • Appropriate assessment, treatment, and monitoring of response to therapy. When surgical intervention is deemed necessary in patients at high risk or with complex anatomical abnormalities, such interventions should be performed in a center with a large patient population that has experience in the management of these conditions and usually has a multidisciplinary approach that includes cardiology, thoracic surgery, and rheumatology requires.

In conclusion, coronary artery vasculitis is rare but can be fatal. Knowledge of the clinical features associated with the most common vasculitis, angiographic findings, and appropriate complementary imaging modalities can aid in efficient assessment and diagnosis. Further research is needed to provide guidance on the best long-term approaches to treating clinically significant coronary artery stenosis.


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Keywords: Angioplasty,antineutrophil cytoplasmic antibodies,vasculitis associated with neutrophil cytoplasmic antibodies,arteriesAorta,atherosclerosis,Blutsenkung,Brachiocephalischer Stamm,pathological narrowing,Chills,koronares Aneurysma,Koronarangiographie,coronary heart disease,Koronararterien-Bypass,coronary restenosis,coronary arteries,coronary stenosis,C-reactive protein,drug eluting stents,Erdheim-Chester Disease,Riesenzellarteriitisendarteriectomy,Inflammation,Diabetes mellitus, Type 2magnetic resonance spectroscopy,magnetic resonance imaging,immunoglobulin G,mammary artery,mucocutaneous lymph node syndrome,myocardial reperfusion,myocarditis,neoplasms,microcirculation,Probability,percutane Coronary intervention,pericarditis,nuclear medicine,positron emission tomography,Polyarteritis nodosaPhysical examination,Forecast,prospective studies,First aid,forwarding and advice,retrospective studies,Rheumatology,risk factors,Arterita de Takayasu,breast surgery,systemic vasculitis,computed tomography, radiography,weight loss

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