Clinical forms and symptom complexes
The disease is divided into two variants that differ cardinally in localization and symptomatology:
- «Classical» polyarteritis nodosa (ICD-10: M30.0). It affects visceral arteries and is rare in children. The key manifesting sign is the sudden development of severe arterial hypertension (AH). Abdominal syndrome (up to intestinal necrosis) and multiple mononeuropathy are also characteristic. A hepatitis B virus marker is frequently detected.
- Juvenile polyarteritis (ICD-10: M30.2). It debuts in early school age (7–10 years), occasionally in preschoolers. It affects peripheral arteries.
The symptom complex of the juvenile form includes:
- Skin changes: livedo reticularis (livedo reticularis), intradermal/subcutaneous nodules, local edema.
- Neurology: peripheral polyneuropathy or mononeuritis. One of the manifestations is marked hyperesthesia.
- Pain syndrome: paroxysmal, burning, and bursting pains in the area of large joints and distal extremities (severe myalgia and arthralgia).
Onset features and complications
The onset of the disease is most often acute. Daily fever (38–39 °C), profuse sweating, rapid exhaustion (dystrophy), and weight loss come to the forefront. A few weeks later, muscle and joint pain joins, forming the full-blown clinical picture. Less commonly, the onset is subacute, when specific symptoms develop insidiously over several months.
The main danger of juvenile polyarteritis is thromboangiitic syndrome. It develops in approximately half of the patients and leads to severe ischemia. Without treatment, the process rapidly progresses to skin necrosis and can result in pregangrene or gangrene of the fingers and toes.
For the classical form, the main life-threatening complications are acute cerebrovascular accidents (stroke) and peritonitis. The prognosis of the classical variant is significantly more severe, up to a fatal outcome.
Diagnostic approach
The basis for establishing a diagnosis is the identification of specific clinical symptom complexes.
Laboratory markers are nonspecific, but during an exacerbation, they clearly reflect systemic inflammation: hyperleukocytosis, hypochromic anemia, and a significant increase in the erythrocyte sedimentation rate (ESR) are recorded. In patients with the classical variant, surface antigen (HBsAg) and other markers of the hepatitis B virus are detected in the blood. These markers may be detected in isolation, without changes in liver function tests or clinical signs of active hepatitis.
Differential diagnosis:
- Classical form: the diagnosis is often established by exclusion. It is necessary to differentiate from other causes of AH (renal artery stenosis, pheochromocytoma, primary renal pathologies).
- Juvenile form: requires the exclusion of sepsis, dermatomyositis, and rheumatoid arthritis.
Principles of therapy
The global goal of treatment is to suppress immunopathological inflammation of the vascular wall and restore blood circulation in ischemic areas.
- Drug base for the juvenile form: Drugs of choice are glucocorticosteroids (prednisolone, methylprednisolone). In the active phase, 1–1.5 mg/kg/day is prescribed for 1–1.5 months, then the dose is gradually reduced to a maintenance dose. The general course of therapy lasts for at least two years. With adequate treatment, the prognosis is favorable (10-year survival is 100%).
- Classical variant: Requires combined immunosuppression. Cytostatics (cyclophosphamide in intravenous pulse therapy regimens: 500 mg/m² once a month) are used along with low maintenance doses of prednisolone (0.3–0.5 mg/kg per day).
- Concomitant therapy: Anticoagulants (heparin) and antiplatelet agents (pentoxifylline, dipyridamole) are used to save microcirculation. If HBV is present, antiviral drugs must be included, and in case of high AH, antihypertensive agents are used.
Class and exam questions
How does thromboangiitic syndrome manifest?
This is a severe complication in which ischemia of the distal extremities occurs. It manifests as rapid skin necrosis and, in severe cases, leads to gangrene of the fingers and toes.
What is the main symptom of the onset of the classical form in children?
The key manifesting sign of «classical» polyarteritis is the sudden development of severe arterial hypertension, which is why the diagnosis is often made after excluding other causes of AH.
What are the features of pain syndrome in juvenile vasculitis?
Patients complain of severe, paroxysmal pain with a sensation of burning and bursting. The pain is localized predominantly in the periphery of the extremities and in the area of large joints.
What else to study on this topic
- Mechanisms of development of thromboangiitic syndrome and ischemia management tactics
- Differential diagnosis of the juvenile form from sepsis and dermatomyositis
- Regimens for the gradual reduction of glucocorticosteroid doses (transition to maintenance therapy)
- Criteria for excluding renal artery stenosis and pheochromocytoma in AH
- Protocol for the use of antiviral drugs in HBV-associated vasculitis
Master the entire topic in the Pediatrics course
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- Full guidelines on «Polyarteritis nodosa» and 3,738 more pages of the subject
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Other topics of «Chapter 9. Rheumatic Diseases»
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