Polyarteritis nodosa

Polyarteritis nodosa (Kussmaul-Mayer disease, classic polyarteritis nodosa, polyarteritis nodosa with predominant damage to internal organs, polyarteritis nodosa with predominant damage to peripheral vessels, polyarteritis nodosa with leading thromboangiitis syndrome) is an acute, subacute or chronic disease, which is based on damage to peripheral and visceral arteries, mainly small and medium caliber, the development of destructive-proliferative arteritis and subsequent peripheral and visceral ischemia.

ICD 10 code

• M30.0 Nodular polyarteritis.
• M30.2 Juvenile polyarteritis.

Epidemiology of polyarteritis nodosa

Polyarteritis nodosa is considered a rare disease (registered with a frequency of 0.7-18 per 100,000 population); in the population it occurs more often in men than in women (6:4).

The frequency of polyarteritis nodosa in children is unknown. Currently, classic polyarteritis nodosa is extremely rare; juvenile polyarteritis is more common, characterized by a pronounced hyperergic component with possible formation of foci of necrosis of the skin and mucous membranes and gangrene of the distal extremities. The disease occurs in all periods of childhood, but juvenile polyarteritis most often begins before the age of 7 and in girls.

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Causes of polyarteritis nodosa

The etiology of the disease in most cases remains unknown. Among the predisposing factors for the development of polyarteritis nodosa, the following can be distinguished:

• hepatitis B viruses (most common), hepatitis C, cytomegalovirus, parvovirus, HIV;
• taking medications (antibiotics, sulfa drugs);
• vaccination, serums;
• childbirth;
• insolation.

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Pathogenesis of polyarteritis nodosa

The pathogenesis of vascular damage is associated with the deposition of circulating immune complexes in their wall and the development of cellular immune reactions leading to inflammatory infiltration and activation of the complement system with subsequent development of fibrinoid necrosis, disruption of the structural organization of the arterial wall and the formation of aneurysms. Subsequently, sclerotic processes occur, leading to occlusion of the lumen of the arteries.

What causes polyarteritis nodosa?

Symptoms of polyarteritis nodosa

Polyarteritis nodosa is characterized by polymorphism of clinical manifestations (multiple mononeuritis, focal necrotic glomerulonephritis, cutaneous vascular infarctions, livedo reticularis, arthritis, myalgia, abdominal pain, convulsions, strokes, etc.). Local (monoorgan) cutaneous form of the disease may develop in 10% of patients: palpable purpura, livedo reticularis, ulcers, painful nodules. However, damage to all tissues and organs (less often - vessels of the lungs and spleen) with signs of fibrinoid necrosis, polymorphic cellular infiltration, local destruction of elastic membranes, thrombosis and aneurysms of the vascular wall is more often observed.

Polyarteritis nodosa ranks first among systemic vasculitides in the development of cardiovascular complications, among which coronariitis can be distinguished. The latter is characterized by the prevalence of the distal nature of the lesion, painless forms of ischemia and focal myocardial necrosis with the development of focal myocardial fibrosis, determined at autopsy. Clinically, coronary artery nasculitis can manifest itself as angina pectoris, acute myocardial infarction, sudden cardiac death and chronic heart failure. According to E. N. Semenkova, chronic heart failure is noted in 39% of patients with polyarteritis nodosa.

Fibrinoid pericarditis may develop, but in these cases it is always necessary to exclude uremia. Characteristic is arterial hypertension (AH), associated with an increase in the content of renin in the blood (hyperrenin form of AH), which, in turn, is associated with the involvement of the renal vascular bed in the process and the formation of ischemic kidney disease in some cases. Arterial hypertension is characterized by consistently high blood pressure figures, in a third of cases - a malignant course with rapid damage to target organs, the development of ischemic and hemorrhagic strokes, dilation of the heart cavities and the progression of chronic heart failure.

Symptoms of polyarteritis nodosa

Diagnosis of polyarteritis nodosa

Diagnosis of polyarteritis nodosa before the development of characteristic clinical picture is extremely difficult. Laboratory data do not have deviations specific for polyarteritis nodosa.

• Clinical blood test (mild normochromic anemia, leukocytosis, thrombocytosis, increased ESR).
• Blood biochemistry (increased levels of creatinine, urea, C-reactive protein, as well as alkaline phosphatase and liver enzymes with normal bilirubin levels).
• General urine analysis (hematuria, moderate proteinuria).
• Immunological study (increased levels of circulating immune complexes, decreased C3 and C4 complement components) antibodies to phospholipids (aPL) are detected in 40-55% of patients, which is associated with livedo reticularis, ulcers and skin necrosis, ANCA (three are rarely detected in the classical course of polyarteritis nodosa.
• Detection of HBsAg in the blood serum of patients.

For morphological confirmation of the diagnosis of polyarteritis nodosa, patients are recommended muscle biopsy, as well as biopsy of the kidneys, liver, testicle, gastric mucosa and rectum. If it is impossible to perform or if questionable data are obtained, patients are recommended angiography of the kidneys, liver, gastrointestinal tract and mesentery, which reveals aneurysms from 1 to 5 mm and/or stenosis of medium-sized arteries.

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Diagnostic criteria for polyarteritis nodosa

To diagnose polyarteritis nodosa, the criteria proposed by the American College of Rheumatology in 1990 are used (Lightfoot R. et al., 1990).*

• Weight loss of 4 kg or more since the onset of the disease (not related to dietary factors).
• Livedo reticularis - patchy, mesh-like changes in the skin pattern on the limbs and trunk.
• Pain in the testicles or their tenderness upon palpation, not associated with traumatic injury, infectious process, etc.
• Diffuse myalgia, weakness or pain on palpation in the muscles of the lower extremities.
• Mononeuritis is the development of multiple mononeuropathy or polyneuropathy.
• Development of arterial hypertension with a diastolic blood pressure level >90 mm Hg.
• Increased urea levels >40 mg% and/or creatinine >15 mg% in the blood serum, not associated with dehydration or impaired urine output,
• Hepatitis B virus infection (presence of HBsAg or antibodies to hepatitis B virus in the blood serum).
• Angiographic changes (aneurysms and/or occlusions of visceral arteries not associated with atherosclerosis, fibromuscular dysplasia and other non-inflammatory diseases).
• Biopsy data (neutrophilic infiltration of the walls of medium and small arteries, necrotizing vasculitis).

The presence of three or more criteria allows for a diagnosis of polyarteritis nodosa (sensitivity - 82%, specificity - 87%), however, the diagnosis can only be considered reliable after a biopsy or angiography.

Diagnosis of polyarteritis nodosa

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Treatment of polyarteritis nodosa

Patients with polyarteritis nodosa are prescribed glucocorticoids. According to indications, pulse therapy with methylprednisolone can be used. Due to the high frequency of disease progression, monotherapy with glucocorticoids may be ineffective. In the absence of markers of hepatitis B virus replication, it is necessary to consider including daily oral cyclophosphamide in the treatment program. In the presence of markers of hepatitis B virus replication, interferon-alpha and ribavirin in combination with glucocorticoids and plasmapheresis are indicated.

How is polyarteritis nodosa treated?

How to prevent polyarteritis nodosa?

Primary prevention of polyarteritis nodosa has not been developed. Prevention of exacerbations and relapses consists of preventing exacerbations and sanitizing infection foci.

Prognosis of polyarteritis nodosa

Nodular polyarteritis has a relatively favorable prognosis. Five-year survival is about 60%. Factors that worsen the course of the disease and, accordingly, the prognosis include: age over 50 years, development of cardiomyopathy, damage to the central nervous system, kidneys and gastrointestinal tract. The cause of death is the consequences of damage to the cardiovascular system.

History of the issue

A detailed clinical and morphological picture of polyarteritis nodosa was first described in 1866 by A. Kussmaul and R. Maier, who observed a 27-year-old man with febrile fever, myalgia, productive cough, protsinuria, abdominal syndrome, and who died 1 month after the onset of the first symptoms of the disease. At autopsy, multiple aneurysms were found in the arteries of medium and small caliber of the muscular type.

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