Symptoms of nodular polyarteritis

The acute symptoms of nodular polyarteritis are characterized by the following symptoms: fever, joint pain, muscle pain, and various combinations of typical clinical syndromes - cutaneous, thrombangiotic, neurological, cardiac, abdominal, renal.

[1], [2], [3],

Common symptoms of nodular polyarteritis

Nodular polyarteritis in most children begins acutely: the body temperature rises, which reaches 38-39 ° C every day for several weeks, dropping down to normal figures after several hours, which is followed by profuse sweat and growing dystrophy. Later there are pains in the joints, muscles and signs of systemic vascular lesions. Less often the disease develops slowly, subacute. In this case, the patient continuously notes weakness, malaise, subfebrile condition or unmotivated short-term body temperature rises. Typical clinical syndromes appear only after a few months. The sequence of the occurrence of syndromes, the variants of their combination are different, which explains the polymorphism of the clinical picture of nodular polyarteritis.

Skin syndrome in children is much more common than in adults. Typical skin changes are considered to be lyvedo, subcutaneous or intradermal nodules and local edema. Livedo (persistent cyanotic spots in the form of branches of the tree or pronounced marbling) occurs in most patients, is located more often on the distal parts of the extremities, changing the color from bright to pale during remission. Single or multiple nodules palpate along the course of large vessels and in a network of livedo. Against the backdrop of treatment, they disappear after a few days. Painful dense edema is localized on the hands, feet, in the region of the joints; As the disease develops, they can disappear, or in their place, necrosis of the skin is formed.

Thrombangiotic syndrome develops in almost every third patient with juvenile polyarteritis and is manifested by the rapid formation of necrosis of the skin and mucous membranes, gangrene of distal limbs. The process is accompanied by severe paroxysmal pains, as well as burning and flaring in the area of large joints and distal limbs. After the appointment of adequate therapy, the pains cease, the foci of skin necrosis and dry gangrene of the terminal phalanges are gradually mummified and demarcated. Along with skin necrosis and distal gangrene, wedge-shaped necrosis of the tongue is often observed. Epithelization and healing of skin and mucous membrane necrosis occur in a few days, mummified phalanges are rejected not earlier than the second month from the start of treatment.

Neurological syndrome. The defeat of the peripheral nervous system in the form of asymmetric polyneuritis (multiple mononeuritis) is a characteristic feature of classical nodular polyarteritis. Polyneuritis develops sharply with hyperesthesia in the affected area; then severe pains appear like a causalgia, and after a few hours or weeks paralysis of various parts of the limbs develops. With juvenile polyarteritis, the defeat of the peripheral nervous system is usually manifested by a picture of mononeuritis. With the onset of remission, movements in the affected limbs are restored. In addition, all the patients in the active period identify symptoms of the defeat of the autonomic nervous system: progressive weight loss, profuse sweating, causalgic nature of pain.

Abdominal syndrome. Patients often observe paroxysmal pains in the abdomen, which can be accompanied by dyspeptic phenomena, palpatory tenderness along the bowel without signs of irritation of the peritoneum, moderate enlargement of the liver. Pain and dyspeptic phenomena quickly pass against the background of pathogenetic therapy. In severe cases, single or diffuse ulcers, necrosis or gangrene of the intestine, including appendicitis, may develop.

Renal syndrome with renal hypertension occurs with classical nodular polyarteritis. Special attention should be paid to the syndrome of arterial hypertension characteristic for this disease, caused by ischemia of the juxtaglomerular apparatus of the kidneys with a violation of the mechanism of renin-angiotensin-aldosterone. Changes in urinary sediment in such cases are insignificant (moderate trace proteinuria and microhematuria), the functional state of the kidneys is not disturbed. The severity of the condition of children and the seriousness of the prognosis are due to a persistent increase in blood pressure, sometimes reaching values of 220 / 110-240 / 170 mmHg. It is these patients who often observe a fatal outcome. With a positive effect of therapy, a remission occurs. The level of arterial pressure decreases, changes in urinary sediment disappear.

Cardiac syndrome at the height of activity is observed in many patients, but it does not determine the main severity of nodular polyarteritis. Clinically, patients note the expansion of the heart, pronounced tachycardia, systolic murmur over the heart. With instrumental studies, you can identify signs of coronary circulation, conduction, changes in the heart muscle, pericardial effusion, in the case of arterial hypertension - myocardial hypertrophy. Against the background of pathogenetic therapy, ischemic and inflammatory changes in the heart disappear.

Pulmonary syndrome in most children has no clinical manifestations, it is detected only when X-ray examination in the form of strengthening the vascular pattern, the hardness of the roots, sometimes in the form of adhesive processes in the pleural sheets, i.e. Pictures of pneumonitis.

Common manifestations of nodular polyarteritis and juvenile polyarteritis are similar, but local symptoms and syndromes are different due to the predominant vascular pathology (internal organs - with classical nodular polyarteritis, peripheral vessels - with juvenile polyarteritis).

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

The course of nodular polyarteritis

The course of nodular polyarteritis can be acute, subacute and chronic (relapsing). In acute course, a short initial period and rapid generalization of vascular lesions are observed. Subacute flow has a gradual onset, it is often noted with classical nodular polyarteritis, signs of disease activity are held for 1-2 years. Nodular polyarteritis associated with the hepatitis B virus, occurs with the syndrome of arterial hypertension (often malignant), is characterized by a more acute onset and often unfavorable course. Chronic course with alternation of exacerbations and remissions occurs mainly with juvenile polyarteritis. In the first years of exacerbation occur after 0.5-1.5 years, after 2-4 exacerbations, remission may extend to 5 years or more.

Clinic of nodular polyarteritis depending on the primary localization of vasculitis

Clinical variant	Leading clinical symptoms and active phase syndromes
Classical	Arterial hypertension of renal genesis. Multiple mononeuritis. The coronaritis. Isolated or generalized necrotic lesion of the intestine. Markers of the hepatitis B virus
Juvenile	Pain in the joints, muscles, hyperesthesia. Nodules, livedo, local edema, polyneuritis. Thrombangiotic syndrome - foci of skin necrosis, mucous membranes, distal gangrene