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Systemic vasculitis
Last reviewed: 07.07.2025

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Systemic vasculitis is a heterogeneous group of diseases based on immune inflammation and necrosis of the vascular wall, leading to secondary damage to various organs and systems.
Systemic vasculitis is a relatively rare pathology in humans. There are no epidemiological studies on the incidence of juvenile forms of systemic vasculitis. In scientific and scientific-practical literature, systemic vasculitis is considered in the group of rheumatic diseases. The basis of working classifications of systemic vasculitis proposed by specialists are morphological signs: the caliber of the affected vessels, the necrotizing or granulomatous nature of inflammation, the presence of giant multinucleated cells in granulomas. In ICD-10, systemic vasculitis is included in the heading XII "Systemic connective tissue disorders" (M30-M36) with subsections "Nodular polyarteritis and related conditions" (M30) and "Other necrotizing vasculopathies" (M31).
There is no universal classification of systemic vasculitides. Throughout the history of studying this group of diseases, attempts have been made to classify systemic vasculitides by clinical features, main pathogenetic mechanisms and morphological data. However, in most modern classifications, these diseases are divided into primary and secondary (in rheumatic and infectious diseases, tumors, organ transplants) and by the caliber of the affected vessels. A recent achievement has been the development of a unified nomenclature of systemic vasculitides: at the International Consensus Conference in Chapel Hill (USA, 1993), a system of names and definitions of the most common forms of systemic vasculitides was adopted.
Epidemiology
The incidence of systemic vasculitis in the population ranges from 0.4 to 14 or more cases per 100,000 population.
The main types of heart damage in systemic vasculitis:
- Cardiomyopathies (specific myocarditis, ischemic cardiomyopathy). The incidence rate according to autopsy data is from 0 to 78%. Most often detected in Churg-Strauss syndrome, less often in Wegener's granulomatosis, nodular polyarteritis and microscopic polyarteritis.
- Coronariitis. Manifested by aneurysms, thromboses, dissection and/or stenosis, each of these factors can lead to the development of myocardial infarction. In one of the pathomorphological studies, coronary vessel damage in patients with nodular polyarteritis was found in 50% of cases. The highest incidence of coronary vasculitis was noted in Kawasaki disease, with aneurysms developing in 20% of patients.
- Pericarditis.
- Endocarditis and valve lesions. In the last 20 years, data on specific valve lesions have become more frequent. It is possible that we are talking about an association of systemic vasculitis with antiphospholipid syndrome (APS).
- Conduction system lesions and arrhythmia. Rare.
- Aortic involvement and dissection. The aorta and its proximal branches serve as endpoint targets in Takayasu arteritis and Kawasaki disease, as well as in giant cell arteritis. At the same time, involvement of small vessels, as well as the vasa vasorum of the aorta, occasionally observed in vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA), can lead to the development of aortitis.
- Pulmonary hypertension. Cases of pulmonary hypertension in vasculitis are rare, isolated cases have been noted in polyarteritis nodosa.
- Main cardiovascular manifestations and their frequency in systemic necrotizing vasculitis.
- Cardiomyopathy - up to 78% depending on detection methods (ischemic cardiomyopathy - in 25-30%).
- Coronary artery disease (with stenosis, thrombosis, aneurysm formation or dissection) - 9-50%.
- Pericarditis - 0-27%.
- Damage to the cardiac conduction system (sinus or AV node), as well as arrhythmia (usually supraventricular) - 2-19%.
- Damage to the valves (valvulitis, aseptic endocarditis) is in most cases an exception (although signs of damage to the heart valves can appear in 88% of patients, and in most of them they are caused by non-specific or functional reasons).
- Aortic dissection (proximal branches of the aorta) - in exceptional cases with Wegener's granulomatosis and Takayasu's arteritis.
- Pulmonary hypertension - in exceptional cases.
Recently, along with the degree of activity in systemic vasculitis, the index of damage to organs and systems has also been determined, which is important for predicting the outcome of the disease.
Cardiovascular Damage Index in Cardiac Vasculitides (1997)
Cardiovascular Damage Criteria |
Definition |
Angina or coronary artery bypass grafting |
History of angina, confirmed by at least ECG data |
Myocardial infarction |
History of myocardial infarction confirmed by at least ECG and biochemical tests |
Recurrent myocardial infarction |
Development of myocardial infarction at least 3 months after the first episode |
Cardiomyopathy |
Chronic ventricular dysfunction confirmed by clinical picture and additional examination methods |
Heart valve disease |
Pronounced systolic or diastolic murmur, confirmed by additional research methods |
Pericarditis for more than 3 months or hystericardiotomy |
Exudative or constrictive pericarditis for at least 3 months |
Hypertension (diastolic blood pressure over 95 mmHg) or taking antihypertensive drugs |
Increase in diastolic blood pressure over 95 mm Hg or the need to take antihypertensive drugs |
Depending on the presence or absence of the specified lesions in the patient, 1 or 0 points are given, respectively. The summary system for assessing organ damage reflects the degree of dysfunction of their function against the background of vascular inflammation and/or treatment. Signs of organ damage due to vasculitis should persist in the patient for 3 months. Recurrence of signs of organ damage is considered newly developed if more than 3 months have passed since its first appearance. On average, in patients with vasculitis, the damage index is 3 points. When monitoring the patient, the index can either remain at the same level or increase (up to a maximum of 8).
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Classification of systemic vasculitis according to ICD-10
- MZ0 Polyarteritis nodosa and related conditions.
- F- M30.0 Nodular polyarteritis.
- M30.1 Polyarteritis with pulmonary involvement (Churg-Strauss), allergic and granulomatous angiitis.
- M30.2 Juvenile polyarteritis.
- MZ0.3 Mucocutaneous lymphonodular syndrome (Kawasaki).
- M30.8 Other conditions associated with polyarteritis nodosa.
- M31 Other necrotizing vasculopathies.
- M31.0 Hypersensitivity angiitis, Hutzpasture's syndrome.
- M31.1 Thrombotic microangiopathy, thrombotic and thrombocytopenic purpura.
- M31.2 Lethal median granuloma.
- M31.3 Wegener's granulomatosis, necrotizing respiratory granulomatosis.
- M31.4 Aortic arch syndrome (Takayasu).
- M31.5 Giant cell arteritis with polymyalgia rheumatica.
- M 31.6 Other giant cell arteritis.
- M31.8 Other specified necrotizing vasculopathies.
- M31.9 Necrotizing vasculopathy, unspecified.
In childhood (except giant cell arteritis with rheumatic polymyalgia) various vasculitides may develop, although in general many systemic vasculitides predominantly affect adults. However, in the case of development of a disease from the group of systemic vasculitides in a child, it is characterized by the acuteness of the onset and course, bright manifest symptoms and at the same time - a more optimistic prognosis in conditions of early and adequate therapy than in adults. Three diseases from those listed in the classification begin or develop mainly in childhood and have syndromes different from systemic vasculitides of adult patients, therefore they can be designated as juvenile systemic vasculitides: nodular polyarteritis, Kawasaki syndrome, nonspecific aortoarteritis. Juvenile systemic vasculitis certainly includes Henoch-Schonlein purpura (hemorrhagic vasculitis), although in ICD-10 the disease is classified in the section “Blood diseases” as Henoch-Schonlein allergic purpura.
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