Polyarteritis nodosa (PAN) is a systemic inflammatory disease characterized by necrotizing vasculitis of medium and small arteries. This process adversely affects various organs and tissues, causing their ischemia and functional disorders. PAN often manifests itself in the form of nodular formations on the skin, muscles and internal organs, which is a consequence of vasculitis. It is important to note that this disease belongs to the group of systemic vasculitis and can have serious complications, including damage to the kidneys, lungs, nervous system and other organs. Clinical manifestations of the disease can vary from minor general symptoms to acute conditions requiring immediate intervention.
History of the disease and interesting historical facts
Polyarteritis nodosa was first described in 1941 by the Belgian physician A. Clerfayt, who coined the term "therapeutic polyarteritis". However, a more detailed study of this disease began only in the 1950s thanks to the work of N. Ya. Tyapkin and his team, who studied the clinical and morphological features of UP. Interestingly, at the beginning of the 20th century, other vasculitides, such as granulomatosis with polyangiitis, were sometimes misdiagnosed with this disease. In their practice, doctors used various treatment methods, but most of them were not effective enough, which led to significant research in this area. In the 1990s, it was proposed to use corticosteroids and cytostatics in therapy, which significantly increased the effectiveness of treatment and improved the quality of life of patients.
Epidemiology
Polyarteritis nodosa is quite rare. According to various sources, the incidence in the population ranges from 2 to 10 cases per 100,000 people per year. The disease can occur at any age, but most often it is detected in people aged 40 to 60 years. Mainly, males predominate among those affected; the ratio of men to women is approximately 2:1. It has also been found that in some regions (for example, in South America), the incidence may be significantly higher, which may be associated with certain genetic or environmental factors. According to a study conducted by M. K. Gubanov et al. in 2021, among patients with UP, about 30% cases had concomitant infections, which emphasizes their possible role in the pathogenesis of the disease.
Genetic predisposition to this disease
Genetic predisposition plays an important role in the development of polyarteritis nodosa. Studies have shown that certain genes can increase the risk of the disease. In particular, associations with polymorphisms of the HLA-DRB1, TNF-α, and IL-10 genes have been identified. Possible mutations in these genes can lead to immune dysfunctions, which contributes to inflammation and autoimmune activity. A study conducted by JM Greco and colleagues in 2020 found that the presence of certain HLA alleles is associated with increased morbidity among patients with polyarteritis nodosa. However, it should be noted that genetic predisposition is not the only factor, since the disease has a multifactorial nature.
Risk factors for the development of this disease
There are various risk factors that can contribute to the development of polyarteritis nodosa. The main ones include:
- Infectious agents: infections of viral and bacterial origin such as hepatitis B, C and syphilis.
- Environmental factors: exposure to toxic substances such as pesticides and heavy metals.
- Immune disorders: Having other autoimmune diseases may increase your risk of developing UP.
- Smoking: is a significant risk factor for the development of various vasculitides.
- Age: The incidence increases with age, especially in people over 40 years of age.
Diagnosis of this disease
Diagnosis of polyarteritis nodosa involves a comprehensive approach and takes into account clinical manifestations, laboratory and radiological examination results. The main symptoms of the disease may include:
- Body temperature above 38°C.
- Weakness, fatigue and weight loss.
- Pain in muscles and joints.
- Skin manifestations: nodular lesions and purpura.
Laboratory studies typically show inflammatory markers such as elevated C-reactive protein and red cell sedimentation rate. Radiologic studies, including angiography, may reveal vascular narrowing or occlusion. Differential diagnosis is also important because other diseases such as granulomatosis with polyangiitis and systemic lupus erythematosus can have similar symptoms.
Treatment
Treatment of polyarteritis nodosa includes both general measures and specific therapy. General treatment measures are aimed at relieving symptoms and improving the patient's quality of life. Pharmacological treatment includes corticosteroids, which are the basis of therapy, as well as cytostatics such as methotrexate and azathioprine. In severe forms of the disease, it is possible to use levilizumab, an antibody to CD20, which significantly improves treatment results. Surgery may be indicated if it is necessary to eliminate vascular narrowing or occlusion. An important aspect is also the use of immunosuppressive therapy in combination with symptomatic treatment to reduce the activity of the inflammatory process.
List of drugs used to treat this disease
Among the main drugs used to treat polyarteritis nodosa, the following can be distinguished:
- Prednisolone is a corticosteroid and is the primary drug for initiating therapy.
- Methotrexate is a cytostatic drug used for maintenance therapy.
- Azathioprine is an immunosuppressant used when long-term therapy is needed.
- Levilizumab is an antibody to CD20, effective in severe cases of the disease.
- Ibuprofen and paracetamol for symptomatic treatment of pain.
Disease monitoring
Monitoring of the patient's condition with polyarteritis nodosa includes regular examinations and laboratory tests to assess the activity of the disease and the response to treatment. Control stages include periodic tests (clinical and biochemical), as well as an assessment of the functional state of the affected organs. The prognosis with adequate treatment is quite favorable, but complications are possible, which may include:
- Kidney damage with the development of renal failure.
- Hemorrhagic stroke.
- Nervous disorders and neuritis.
Age-related features of the disease
The course of polyarteritis nodosa has its own characteristics depending on the patient's age. In children, the disease may manifest itself more vividly, often with a more acute onset and pronounced symptoms. In older people, on the contrary, clinical manifestations may be less noticeable, which complicates diagnosis. In this age group, concomitant diseases are also more common, which can significantly complicate treatment and worsen the prognosis.
Questions and Answers
- What is polyarteritis nodosa? Polyarteritis nodosa is a systemic inflammatory disease characterized by necrotizing vasculitis affecting medium and small arteries.
- What are the main symptoms of polyarteritis nodosa? The main symptoms include fever, weakness, weight loss, muscle pain and nodular formations on the skin.
- How is the disease diagnosed? Diagnosis includes clinical manifestations, laboratory tests for inflammatory markers, and radiological studies such as angiography.
- How is polyarteritis nodosa treated? Treatment consists of the use of corticosteroids, cytostatics, and in severe cases, immunosuppressants such as levilizumab.
- What is the prognosis for patients with polyarteritis nodosa? The prognosis is favorable with adequate treatment, but serious complications are possible, especially in advanced forms of the disease.
