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Symptoms of polyarteritis nodosa
Last reviewed: 04.07.2025
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[ General symptoms of polyarteritis nodosa
In most children, polyarteritis nodosa begins acutely: the body temperature rises, reaching 38-39 °C daily for several weeks, dropping to normal levels after a few hours, accompanied by profuse sweating and increasing dystrophy. Later, joint and muscle pain and signs of systemic vascular damage appear. Less often, the disease develops gradually, subacutely. In this case, the patient experiences weakness, malaise, subfebrile temperature or unmotivated short-term increases in body temperature for a long time. Typical clinical syndromes appear only after several months. The sequence of syndromes and their combinations may vary, which explains the polymorphism of the clinical picture of polyarteritis nodosa.
Skin syndrome is much more common in children than in adults. Typical skin changes include livedo, subcutaneous or intradermal nodules, and localized edema. Livedo (persistent cyanotic spots in the form of tree branches or pronounced marbling) is found in most patients, is located more often on the distal parts of the extremities, changing color from bright to pale during remission. Single or multiple nodules are palpated along the course of large vessels and in the livedo network. With treatment, they disappear within a few days. Painful dense edema is localized on the hands, feet, in the area of joints; as the disease progresses, they may disappear, or skin necrosis may form in their place.
Thrombangitic 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 the distal parts of the extremities. The process is accompanied by severe paroxysmal pain, as well as a feeling of burning and distension in the area of large joints and distal parts of the extremities. After the appointment of adequate therapy, the pain stops, the foci of skin necrosis and dry gangrene of the terminal phalanges gradually mummify and demarcate. Along with skin necrosis and distal gangrene, wedge-shaped necrosis of the tongue is often observed. Epithelialization and healing of necrosis of the skin and mucous membranes occur after several days, the mummified phalanges are rejected no earlier than the second month from the beginning of treatment.
Neurological syndrome. Peripheral nervous system damage in the form of asymmetric polyneuritis (mononeuritis multiplex) is a characteristic sign of classic nodular polyarteritis. Polyneuritis develops acutely with hyperesthesia in the affected area; then severe pains of the causalgia type appear, and after several hours or weeks paralysis of various parts of the limbs develops. In juvenile polyarteritis, peripheral nervous system damage usually manifests itself as a picture of mononeuritis. With the onset of remission, movements in the affected limbs are restored. In addition, all patients in the active period show symptoms of damage to the autonomic nervous system: progressive weight loss, profuse sweating, causalgic pain.
Abdominal syndrome. Patients often experience paroxysmal abdominal pain, which may be accompanied by dyspeptic symptoms, palpable pain along the intestines without signs of peritoneal irritation, and moderate liver enlargement. Pain and dyspeptic symptoms quickly disappear with pathogenetic therapy. In severe cases, isolated or diffuse ulcers, necrosis, or gangrene of the intestines, including the appendix, may develop.
Renal syndrome with renal hypertension occurs in classic polyarteritis nodosa. Of particular note is the arterial hypertension syndrome characteristic of this disease, caused by ischemia of the juxtaglomerular apparatus of the kidneys with a disorder of the renin-angiotensin-aldosterone mechanism. Changes in urinary sediment in such cases are insignificant (moderate trace proteinuria and microhematuria), the functional state of the kidneys is not impaired. The severity of the condition of children and the seriousness of the prognosis are due to a persistent increase in blood pressure, sometimes reaching 220/110-240/170 mm Hg. It is in these patients that a fatal outcome is often observed. With a positive effect of therapy, 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 have enlarged heart borders, pronounced tachycardia, systolic murmur over the heart area. Instrumental studies can reveal signs of impaired coronary circulation, conduction, changes in the heart muscle, pericardial effusion, and in the case of arterial hypertension - myocardial hypertrophy. Against the background of pathogenetic therapy, ischemic and inflammatory changes in the heart disappear.
In most children, pulmonary syndrome has no clinical manifestations; it is detected only by X-ray examination in the form of increased vascular pattern, stringy roots, and sometimes in the form of adhesive processes in the pleural sheets, i.e., a picture of pneumonitis.
The general manifestations of polyarteritis nodosa and juvenile polyarteritis are similar, but local symptoms and syndromes are different due to the predominant vascular pathology (internal organs - in classical polyarteritis nodosa, peripheral vessels - in juvenile polyarteritis).
The course of polyarteritis nodosa
The course of polyarteritis nodosa may be acute, subacute and chronic (recurrent). In the acute course, a short initial period and rapid generalization of vascular lesions are observed. The subacute course has a gradual onset, it is more often observed in classical polyarteritis nodosa, signs of disease activity persist for 1-2 years. Nodular polyarteritis associated with the hepatitis B virus occurs with arterial hypertension syndrome (often malignant), is characterized by a more acute onset and often an unfavorable course. Chronic course with alternating exacerbations and remissions occurs mainly in juvenile polyarteritis. In the first years, exacerbations occur after 0.5-1.5 years, after 2-4 exacerbations, remission can extend to 5 years or more.
Clinical presentation of polyarteritis nodosa depending on the predominant localization of vasculitis
Clinical variant |
Leading clinical symptoms and syndromes of the active phase |
Classical |
Arterial hypertension of renal genesis. Multiple mononeuritis. Coronaritis. Isolated or generalized necrotic bowel lesion. Hepatitis B virus markers |
Juvenile |
Joint pain, muscle pain, hyperesthesia. Nodules, livedo, local edema, polyneuritis. Thrombangitic syndrome - foci of skin necrosis, mucous membranes, distal gangrene |