Periarteritis nodosa is an immunopathological vascular inflammation. Pathology proceeds with a predominant lesion of small and medium caliber arteries. Symptoms of periarteritis nodosa are very diverse, which makes diagnosis difficult. Untimely therapy leads to serious complications, the prognosis for the treatment of which in most cases is unfavorable.
General information about the disease
A disease characterized by inflammatory-necrotic lesion of peripheral, small and medium visceral arteries is called nodular periarteritis in medicine. Treatment and symptoms (the photo shows the affected area) depends on the age of the patient and the characteristics of the body. Pathology has another name - Kussmaul-Mayer disease.
The disease is characterized by damage to muscle type arteries with the formation of vascular aneurysms ("nodules"), hence the name. The pathological process affects not only the outer shell of the vessel, it extends to all layers of the vascular wall. Inflammatory changes are systemic in nature - vessels and arteries of different levels and caliber are affected. However, the arteries of the muscular and muscular-elastic type are still more affected.
Periarteritis nodosa (photo in the article) is a rare disease. However, there is a clear trend towards its spread. Contributes to this blur etiology and the absence of specific clinical signs. Epidemiology is poorly studied, 1 case per 100 thousand population is recorded per year. Most often, the disease occurs in men aged 30 to 50 years.
Causes of pathology
The etiology of the disease has not been established for certain. The most common cause of periarteritis nodosa (symptoms - indirect evidence of this) is allergy. The most common hyperergic vascular reaction occurs on penicillins, iodine preparations, sulfonamides, chlorpromazine, mercury, the introduction of foreign serums. When examining biomaterial for biopsy during an exacerbation of the disease, antibodies to the allergens immunoglobulins IgG, IgA, IgM are found in the basement membranes of the vascular loops.
There is also every reason to believe that the cause of the development of the pathological process is a viral infection. Most often, it provokes the development of the inflammatory process of arteries, serum hepatitis. Hepatitis B antibodies are found in the walls of the affected arteries and muscles. In 40% of cases of periarteritis nodosa, prolonged conservation of the HBsAg virus is observed.
There was a hypothesis that arteries are affected after an acute illness, the causative agent of which is staphylococcus or streptococcus. The essence of the theory was based on the fact that the pathogen exerts an indirect toxic effect on blood vessels. But in patients with periarteritis, it was not possible to detect gram-positive cocci.
In addition to the main reasons, there are risk factors:
- Constantly elevated blood pressure.
- Congenital weakness of the elastic layer of blood vessels.
- Weakening of the walls of blood vessels due to toxic effects (alcohol, drugs).
- Irradiation by solar radiation.
- Hypothermia.
- Preventive vaccinations.
Pathology classification
There is no generally accepted systematization for a disease. Depending on the location of the affected vessels and symptoms, periarteritis nodosa (photo above) is classified as follows:
- Classic, or polyvisceral. It is accompanied by fever, severe exhaustion, muscle and joint pain. The prognosis for the clinical variant is often not favorable. But rational prevention can significantly extend the life of the patient.
- Asthmatic, or eosinophilic. In many foreign countries, the disease is called allergic angiitis or Cherg-Strauss syndrome. In the initial stages, asthma attacks occur, which are usually preceded by hypersensitivity to drugs, hence the name.
- Cutaneous thromboangiitis. The main symptoms of periarteritis nodosa are skin lesions with the formation of vascular aneurysms, the size of lentils, painful on palpation. In some cases, necrosis of soft tissues, mucous membranes, gangrene of the extremities is observed. The skin variant of the disease is considered the least dangerous.
- Monoorgan. This option is very rare, as a rule, one organ is affected: a kidney, gall bladder. The diagnosis is made only with the histology of the removed affected organ or in the study of biomaterial taken from an organ biopsy.
Also, pathology is classified by the speed of the course:
- Slow progression is characteristic of the cutaneous variant of Kussmaul-Mayer disease. It is characterized by frequent relapses.
- Rapid progression is associated with kidney damage and hypertension. Sometimes the pathology develops at lightning speed, the patient dies after a year.
How does periarteritis nodosa develop?
Using histological and histochemical studies, it was found that morphological changes in the vessels with periarteritis occur in a certain sequence.
First, mucous degeneration of the walls of the vessels occurs. Due to the separation of protein-polysaccharide complexes of connective tissue into parts, vascular permeability increases. Further fibrous necrosis occurs, characterized by prolapse of fibrin masses into the walls of the arteries. Against this background, an inflammatory reaction of cells develops, in which the walls of blood vessels by lymphocytes, leukocytes. Also in the infiltrate fibroblasts, plasmocytes, which very quickly become dominant, are detected, which leads to sclerosis of the walls of the arteries.
The disease develops immediately or sequentially in many organs. But the kidneys, blood vessels of the heart, brain, and intestines are most often affected by nodular periarteritis. In arteritis-affected tissues and organs, local changes occur:
- Hemorrhages - hemorrhages of varying severity.
- Violation of cellular metabolism of the parenchyma.
- The formation of ulcers and necrosis in the affected foci.
- Heart attacks with scarring.
- Violation of the elasticity of blood vessels.
- Cirrhotic changes.
The prevalence of vascular inflammation and the severity due to the process of secondary changes are very diverse, which indicates the disease as polymorphic.
Adult Periarteritis Nodosa: Symptoms
The disease begins with common clinical manifestations. Regardless of which organ is affected, the characteristic signs of vascular inflammation are fever, muscle-joint pain and weight loss. Due to the great variety of symptoms, nodular periarteritis is combined into syndromes that determine the specifics of the clinical picture.
Renal syndrome occurs in approximately 90% of patients and is characterized by the following symptoms:
- Stable arterial hypertension.
- Retinal disease followed by loss of vision.
- Detection of urine protein, red blood cells.
- Rupture of the vessel of the kidney.
- Renal failure develops within three years.
Abdominal syndrome is more often observed at the beginning of the pathology:
- Constant, growing pains in the abdomen.
- Diarrhea, stool frequency up to 10 times a day.
- Anorexia develops due to nausea.
- Inflammation of the peritoneum.
- As a result of rupture of ulcers, stomach bleeding occurs. Such symptoms seriously complicate the treatment of periarteritis nodosa.
- If the cause of the development of the pathology is serum hepatitis, the development of chronic forms of hepatitis and cirrhosis of the liver is often observed.
Cardiac syndrome occurs in approximately 70% of patients:
- Small focal myocardial infarction.
- The proliferation of connective tissue in the heart muscle.
- Heart Rate Disorder.
- Heart failure.
Pulmonary syndrome manifests itself in half of patients with periarteritis:
- Bronchial asthma.
- Cough with a small amount of sputum mucosa, sometimes blood.
- Temperature rise.
- Increasing signs of respiratory failure.
Neurological Syndrome:
- Damage to the peripheral nerves.
- Muscle soreness.
- Muscle weakness.
- Sore muscles, mainly gastrocnemius.
Features of periarteritis nodosa in children
There are fewer small patients suffering from immunopathological vascular inflammation than adults. And this is perhaps the only plus. Girls and boys get sick with the same frequency and at any age.
Symptoms of periarteritis nodosa in children are basically the same as in adults:
- High fever, difficult to treat.
- Growing weakness.
- Weight loss. For children, even slight weight loss can cause serious problems.
- Muscle pains.
- Cyanotic color of the palms and soles.
- Necrosis of the skin mainly on the arms and legs.
- Swelling of the mucous membranes.
In children, classic or cutaneous-thromboangiotic variants of periarteritis nodosa often occur (photo above). Moreover, cutaneous occurs mainly in preschool children. In addition to the general symptoms, painful nodules up to 1 cm in diameter appear on the skin of the legs, which quickly spread to the body.
The course of the pathology is progressive, with severe damage to the heart, kidneys, liver and other organs. Microinfarctions of the internal organs are often asymptomatic, neither the child nor the parents are even aware of the violations.
It is difficult to make a lifetime diagnosis for children. Basically, doctors focus on the most pronounced signs relating to a particular organ.
How is the diagnosis
The set of diagnostic measures for children and adults is the same.
Laboratory tests include general and specific studies:
- In the general blood test, there is a significant excess of neutrophils, ESR. In some cases, there is an excess of the number of eosinophils, a decrease in red blood cells and hemoglobin.
- Analysis for antibodies to immunoglobulins E shows a positive result.
- With hepatitis B ELISA, antibodies to HBsAg are detected in the blood serum.
- A general urinalysis reveals the presence of protein up to 3 g, red blood cells, albumin, creatine.
- With a coprogram in the feces, mucus and impurities of the blood are detected.
The basis for the diagnosis of periarteritis nodosa is the clinical symptoms:
- Weight loss of 4 kg or more with constant nutrition.
- Vascular changes on the skin (cyanotic distinct pattern).
- Pain in the muscles of the legs, and painkillers are ineffective in its elimination.
- Testicular pain not related to trauma or infectious disease.
- Patients complain of constant high blood pressure.
- The characteristic symptoms of mononeuritis are observed: deterioration of muscle sensitivity, inability to bend fingers into a fist, foot, muscles atrophy.
If any three criteria are identified, nodular periarteritis is diagnosed.
Differential diagnosis
Due to the similarity of many diseases with periarteritis nodosa in the symptoms, the treatment of the disease is often prescribed incorrectly. Especially dangerous is antibiotic therapy that worsens the condition of patients. In order to avoid severe consequences, immunopathological vascular inflammation should be differentiated from other diseases:
- Periarteritis must be differentiated from some forms of tumors. Hypernephroma of the kidney, pancreatic cancer occur with similar symptoms. All diseases are characterized by fever, myalgia, a sharp decrease in weight.
- At the very beginning, the clinical picture of vascular inflammation is similar to infectious endocarditis (inflammation of the lining of the heart) and malignant granuloma. Of the common symptoms, chills, profuse sweating and itching are distinguished.
- Clinical abdominal forms are similar to dysentery and an acute abdomen.
In children, the differential diagnosis of Kussmaul-Mayer disease includes the following diseases:
- Lymphogranulomatosis.
- Acute Leukemia
- Infections of viral and bacterial etiology.
- Sepsis.
- Lupus erythematosus.
- Dermatomyositis.
The greatest difficulty is the differentiation of the abdominal syndrome with necrotic enterocolitis, hepatitis, intestinal infections.
Non-drug therapies
In the treatment of periarteritis nodosa, clinical recommendations are as follows:
- Therapeutic measures should be carried out under the constant supervision of medical personnel and the attending doctor. Both adults and children in acute periods of pathology should be in a hospital.
- During the period of exacerbation, the patient's motor mode is limited. Correct posture should be maintained when walking or when the patient is sitting. You need to sleep on a hard mattress and a small thin pillow.
- Exclude mental and emotional stress.
- Showing short daily walks in the evening. Sun exposure should be excluded.
- Due to the immunopathological mechanism of the disease, all patients are required to follow a hypoallergenic diet. With significant progressive weight loss, a protein diet is indicated. In renal syndrome, patient fluid intake is controlled.
- For the prevention of osteoporosis, it is recommended to consume foods high in calcium and cholecalciferol (vitamin D).
- Physiotherapy exercises are carried out depending on the conditions of the patient and his individual capabilities.
Surgical methods are rarely used. The main methods of surgical treatment are prosthetics, bypass surgery (mainly surgery is performed on the heart, less often on the stomach), kidney transplantation.
Drug treatment
Each patient has different symptoms of periarteritis nodosa. Treatment, respectively, is prescribed individually. However, the general methods of therapy are the same for all.
Patients should be well aware that the disease is severe and a positive effect can be achieved only with prolonged continuous, complex therapy. As a rule, treatment is carried out jointly by a rheumatologist, nephrologist, in children - a pediatrician and other specialists.
The most effective are the following drugs:
- Glucocorticosteroid hormones. “Prednisolone”, “Triamphinalon”, “Decortin” are prescribed in large doses, which are reduced, based on therapeutic results. The drugs have anti-inflammatory, immunosuppressive, anti-allergic effects.
- Cytostatics. With the simultaneous use of antitumor drugs and glucocorticoids, the effectiveness of treatment increases to 84%. The most commonly prescribed drugs: Cyclophosphate, Azathioprine, Chlorbutin.
- Angioprotectors are prescribed to reduce platelet aggregation, reduce leukocyte activity (Trental, Dipyridamole).
- When an infection is detected, antibiotic therapy is performed. Drugs are selected individually, based on the resistance of the infectious agent.
- Pain syndromes are eliminated with the help of analgesics, NSAIDs, antispasmodics.
Disease prevention
With the classic version of the disease, the prognosis is poor. But adequate therapy and rational prevention can significantly extend the life of the patient. There are no specific prophylactic recommendations for periarteritis nodosa. It is necessary to respond to the immunopathological reactions of the body when exposed to any factors, especially drugs. Vaccination and blood transfusion is best done in medical facilities where epidemiological surveillance is regularly conducted.