Systemic vasculitis

Systemic vasculitis is a heterogeneous group of diseases, which are 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 human pathology. Epidemiological studies on the incidence of juvenile forms of systemic vasculitis do not. In scientific and scientific-practical literature systemic vasculitis is considered in the group of rheumatic diseases. The basis of the working classifications of systemic vasculitis offered by specialists is the morphological features: the caliber of the affected vessels, the necrotizing or granulomatous nature of the inflammation, the presence of giant multinucleate cells in granulomas. In ICD-10 systemic vasculitis was included in the XII section "Systemic lesions of connective tissue" (M30-M36) with subsections "Nodular polyarteritis and related conditions" (MZ0) and "Other necrotizing vasculopathies" (M31).

There is no universal classification of systemic vasculitis. Throughout the history of the study of this group of diseases, attempts have been made to classify systemic vasculitis according to clinical features, basic pathogenetic mechanisms and morphological data. However, in most modern classifications these diseases are divided into primary and secondary (for rheumatic and infectious diseases, tumors, organ transplantation) and the caliber of affected vessels. The latest achievement was the development of a unified nomenclature of systemic vasculitis: at the International Conciliation Conference in Chepel Hill (USA, 1993), a system of names and definitions of the most common forms of systemic vasculitis was adopted.

Epidemiology

The incidence of systemic vasculitis in the population varies from 0.4 to 14 or more cases per 100 000 population.

The main variants of heart failure in systemic vasculitis:

• Cardiomyopathy (specific myocarditis, ischemic cardiomyopathy). The incidence of autopsy data is from 0 to 78%. Most often found in the syndrome of Chard-Strauss, less often - with Wegener's granulomatosis, nodular polyarteritis and microscopic polyarteritis.
• Coronarians. Are aneurysms, thrombosis, stratification and / or stenosis, and each of these factors can lead to the development of myocardial infarction. In one of the pathomorphological studies, coronary artery disease in patients with nodular polyarteritis was found in 50% of cases. The highest incidence of coronary vasculitis was observed in Kawasaki disease, with aneurysms developing in 20% of patients.
• Pericarditis.
• Endocarditis and valve lesions. In the past 20 years, there have been more frequent reports of specific damage to the valves. It may be an association of systemic vasculitis with antiphospholipid syndrome (APS).
• Disorders of the conduction system and arrhythmias. They are rare.
• The defeat of the aorta and its dissection. The aorta and its proximal branches serve as the target endpoints in Takayasu's arteritis and Kawasaki's disease, as well as in the giant cell arteritis. At the same time, the lesion of small vessels, as well as vasa vasorum of the aorta, occasionally noted in vasculitis associated with antineutrophilic cytoplasmic antibodies (ANCA), can lead to the development of aortitis.
• Pulmonary hypertension. Cases of pulmonary hypertension in vasculitis are rare, isolated cases are noted with nodular polyarteritis.
• The main cardiovascular manifestations and their frequency with systemic necrotizing vasculitis.
• Cardiomyopathy - up to 78% depending on the methods of detection (ischemic cardiomyopathy - in 25-30%).
• The defeat of the coronary arteries (with stenosis, thrombosis, the formation of aneurysms or stratification) is 9-50%.
• Pericarditis - 0-27%.
• Damage to the conduction system of the heart (sinus or AV node), as well as arrhythmias (often supraventricular) is 2-19%.
• Valve damage (valvulitis, aseptic endocarditis) is in most cases an exception (although signs of heart valve damage can appear in 88% of patients, with most of them caused by nonspecific or functional reasons).
• Stratification of the aorta (proximal aortic branches) - 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 is also determined, which is important for predicting the outcome of the disease.

Index of damage to the cardiovascular system in cardiac vasculitis (1997)

Criteria for damage to the cardiovascular system	Definition
Angina pectoris or coronary artery bypass graft	Angina in history, confirmed by at least ECG data
Myocardial infarction	Myocardial infarction in anamnesis, confirmed by at least ECG data and biochemical tests
Repeated myocardial infarction	The development of myocardial infarction at least 3 months after the first episode
Cardiomyopathy	Chronic ventricular dysfunction, confirmed by clinical picture and data from additional examination methods
Heart valve disease	Severe systolic or diastolic noise, confirmed by data from additional research methods
Pericarditis more than 3 months or gerecardiotomy	Exudative or constrictive pericarditis for at least 3 months
Hypertension (diastolic blood pressure more than 95 mm Hg) or taking antihypertensive drugs	Increased diastolic blood pressure more than 95 mm Hg. Or the need for taking antihypertensive drugs

Depending on the presence or absence of the patient, these lesions are 1 or 0 points, respectively. The total system of damage assessment of organs reflects the degree of disruption of their function against the background of vascular inflammation and / or treatment. Signs of organ damage due to vasculitis should be maintained in the patient for 3 months. Re-emergence of signs of organ damage is considered to have arisen, if after its first appearance more than 3 months have passed. On average, in patients with vasculitis, the damage index is 3 points. When monitoring a patient, the index can either remain at the same level, or increase (up to a maximum of 8).

[1], [2], [3], [4], [5], [6], [7]

Classification of systemic vasculitis according to ICD-10

• MZ0 Nodular polyarteritis and related conditions.
• F-M30.0 Nodular polyarteritis.
• M30.1 Polyarteritis with lung lesions (Czordzha Strauss), allergic and granulomatous angiitis.
• M30.2 Juvenile polyarteritis.
• MZ0.Z Mucous-cutaneous lymphonodular syndrome (Kawasaki).
• M30.8 Other conditions associated with nodular polyarteritis.
• M31 Other necrotizing vasculopathies.
• M31.0 Hypersensitive angiitis, Guzacecher's syndrome.
• M31.1 Thrombotic microangiopathy, thrombotic and thrombocytopenic purpura.
• M31.2 Mortal median granuloma.
• M31.3 Wegener's granulomatosis, necrotizing respiratory granulomatosis.
• M31.4 Syndrome of the aortic arch (Takayasu).
• M31.5 Giant cell arteritis with rheumatic polymyalgia.
• M 31.6 Other giant cell arteritis.
• M31.8 Other specified necrotizing vasculopathies.
• M31.9 Necrotizing vasculopathy, unspecified.

In childhood (with the exception of giant cell arteritis with rheumatic polymyalgia), various vasculitides may develop, although in general many systemic vasculitis are predominantly affected by adults. However, in the case of the development of the disease from the group of systemic vasculitis in the child, it is characterized by the severity of the onset and course, bright manifest symptoms and at the same time a more optimistic prognosis in the conditions of early and adequate therapy than in adults. Three of the diseases listed in the classification begin or develop primarily in childhood and have excellent syndromes from systemic vasculitis in adult patients, and can therefore be referred to as juvenile systemic vasculitis: nodular polyarteritis, Kawasaki syndrome, nonspecific aortoarteritis. To juvenile systemic vasculitis is clearly referred to as purple Shenlaine-Genocha (hemorrhagic vasculitis), although in ICD-10 the disease is classified in the section "Diseases of the blood" as an allergic purpura of Shenlaine-Genoch.

[8], [9], [10]