Nodular polyarteritis

Nodular polyarteritis (Kussmaul-Mayer disease, classic nodular polyarteritis, nodular polyarteritis with predominant lesion of internal organs, nodular polyarteritis with predominant involvement of peripheral vessels, nodular polyarteritis with leading thrombangiotic syndrome) - acute, subacute or chronic disease, which is based on damage to peripheral and visceral arteries, predominantly of small and medium caliber, development of destructive-proliferative arteritis and subsequent perforation fericheskoy and visceral ischemia.

ICD Code 10

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

Epidemiology of nodular polyarteritis

Nodular polyarteritis is considered a rare disease (recorded at a frequency of 0.7-18 per 100 000 population); in the population is more common in men than in women (6: 4).

The frequency of nodular polyarteritis in children is not known. Currently, classical nodular polyarteritis is extremely rare, juvenile polyarteritis is more common, characterized by a pronounced hyperergic component with the possible formation of foci of skin and mucosal necrosis and gangrene of distal limbs. The disease occurs in all periods of childhood, but juvenile polyarteritis often begins before the age of 7 years and in girls.

[1], [2], [3]

Causes of nodular polyarteritis

The etiology of the disease in most cases remains unknown. Among predisposing factors of nodular polyarteritis development we can distinguish:

• hepatitis B viruses (most often), hepatitis C, cytomegalovirus, parvovirus, HIV;
• taking medicines (antibiotics, sulfanilamide preparations);
• vaccination, serums;
• childbirth; 
• insolation.

[4], [5], [6], [7]

Pathogenesis of nodular polyarteritis

The pathogenesis of vascular lesions is associated with the deposition of circulating immune complexes in their wall and the development of cellular immune responses leading to inflammatory infiltration and activation of the complement system, followed by the development of fibrinoid necrosis, a violation of the structural organization of the artery wall and the formation of aneurysms. Subsequently, sclerotic processes occur leading to occlusion of the lumen of the arteries.

What triggers nodular polyarteritis?

Symptoms of nodular polyarteritis

Nodular polyarteritis is characterized by polymorphism of clinical manifestations (multiple mononeuritis, focal necrotic glomerulonephritis, skin vessel infarctions, reticular livedo, arthritis, myalgia, abdominal pain, seizures, strokes, etc.). In 10% of patients, a local (monoorganic) cutaneous form of the disease can develop: palpable purpura, reticular livedo, ulcers, painful nodules. However, damage to all tissues and organs (less often - vessels of the lungs and spleen) with signs of fibrinoid necrosis, polymorphic cell infiltration, local destruction of elastic membranes, thrombosis and aneurysms of the vascular wall is more often observed.

Nodular polyarteritis takes first place among systemic vasculitides for the development of cardiovascular complications, among which coronary artery can be identified. The peculiarity of the latter is the prevalence of the distal nature of the lesion, painless forms of ischemia and focal necrosis of the myocardium with the development of focal fibrosis of the myocardium, determined at autopsy. Clinically, coronary artery swelling can be manifested by angina pectoris, acute myocardial infarction, sudden cardiac death and chronic heart failure. According to E.N. Semenkovoy, chronic heart failure noted in 39% of patients with nodular polyarteritis.

Fibrinoid pericarditis can develop, in these cases it is always necessary to exclude uremia. Arterial hypertension (AH) is associated with an increase in renin content in the blood (hyperenenic form of hypertension), which in turn is associated with the involvement of the kidney and vascular bed in the process and the formation of ischemic kidney disease in a number of cases. Arterial hypertension is characterized by consistently high BP numbers, in a third of cases - malignant course with rapid damage to target organs, development of ischemic and hemorrhagic strokes, dilated heart cavities and progression of chronic heart failure.

Symptoms of nodular polyarteritis

Diagnosis of nodular polyarteritis

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

• Clinical blood test (normochromic anemia of mild degree, leukocytosis, thrombocytosis, increased ESR).
• Biochemical blood test (increase in creatinine, urea, C-reactive protein, as well as alkaline phosphatase and liver enzymes at a normal level of bilirubin).
• General analysis of urine (hematuria, moderate proteinuria).
• Immunological studies (increasing the level of circulating immune complexes, reducing the C3 and C4 complement components) of antibodies to phospholipids (asplatinum) are found in 40-55% of patients, which is associated with reticular livedo, ulcers and necrosis of the skin, ANCA (three classic nodular polyarteritis .
• Detection in the serum of patients with HBsAg.

For morphological confirmation of the diagnosis of nodular polyarteritis, patients are shown muscular biopsy, as well as biopsy of the kidneys, liver, testicle, gastric mucosa and rectum. When it is impossible to conduct it or obtain questionable data, patients are shown angiography of the kidneys, liver, gastrointestinal tract and mesentery, in which aneurysms from 1 to 5 mm and / or stenoses of arteries of medium caliber are detected.

[8], [9],

Diagnostic criteria of nodular polyarteritis

For the diagnosis of nodular polyarteritis, the criteria proposed by the American College of Rheumatology in 1990 (Lightfoot R. Et al., 1990) are used. *

• Weight loss of 4 kg or more from the time of onset of the disease (not related to dietary habits).
• Reticulate - spotted, reticular changes in skin pattern on limbs and trunk.
• Pain in the testicles or their soreness in palpation, not associated with traumatic injury, infection, etc.
• Diffuse myalgias, weakness or soreness in palpation in the muscles of the lower extremities.
• Mononeuritis - the development of multiple mononeuropathy or polyneuropathy.
• The development of arterial hypertension with a diastolic blood pressure level> 90 mm Hg.
• Increased urea levels> 40 mg% and / or creatinine> 15 mg% in serum, not associated with dehydration or impaired urinary excretion,
• Infection with hepatitis B virus (the presence of HBsAg or antibodies to hepatitis B virus in the blood serum).
• Angiographic changes (aneurysms and / or occlusions of the visceral arteries, not associated with atherosclerosis, fibromuscular dysplasia and other non-inflammatory diseases).
• Data biopsy (neutrophilic infiltration of the walls of the middle and small arteries, necrotizing vasculitis).

The presence of three or more criteria makes it possible to diagnose "nodular polyarteritis" (sensitivity - 82%, specificity - 87%), but the diagnosis can be considered reliable only after a biopsy or angiography.

Diagnosis of nodular polyarteritis

[10], [11]

Treatment of nodular polyarteritis

Patients with nodular polyarteritis are assigned glucocorticoids. According to the indications, one can use pulse therapy with methylprednisolone. Due to the high rate of progression of the disease, glucocorticoid monotherapy may be ineffective. In the absence of markers for the replication of the hepatitis B virus, one should think about including cyclophosphamide in the daily treatment program. In the presence of markers of hepatitis B virus replication, interferon-alpha and ribavirin are shown in combination with glucocorticoids and plasmapheresis.

How is polyarteritis nodosa treated?

How to prevent nodular polyarteritis?

Primary prophylaxis of nodular polyarteritis has not been developed. Preventive maintenance of exacerbations and relapses consists in the prevention of exacerbations, sanation of the centers of infections.

Prognosis of nodular polyarteritis

Nodular polyarteritis has a relatively favorable prognosis. The five-year survival rate is about 60%. The factors that worsen the course of the disease and, correspondingly, the forecast, include: age over 50 years, the development of cardiomyopathy, CNS, kidney and gastrointestinal tract damage. The cause of death is the consequences of the defeat of the cardiovascular system.

Background

A detailed clinical and morphological picture of nodular polyarteritis 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 died 1 month after the onset of the first symptoms of the disease . At the autopsy, multiple aneurysms were revealed in the arteries of medium and small caliber muscle type.

[12], [13], [14], [15], [16], [17], [18]