Periarteritis nodosa reveals itself to be common non-specific manifestations: a person has a constantly elevated temperature, he is progressively losing weight, pain in muscles and joints is disturbing.
An increase in temperature in the form of persistent fever is characteristic of 98-100% of cases: the temperature curve is of the wrong type, there is no response to antibiotic therapy, but corticosteroid therapy is effective. The temperature may subsequently normalize, against the background of the development of multiple organ pathology.
The emaciation of patients is pathognomic. In some patients, weight is reduced by 35-40 kg within a few months. At the same time, the degree of thinness exceeds that in oncopathologies.
Pain in the muscles and joints is especially characteristic of the initial stage of nodular periarteritis. Soreness especially often affects large joints and calf muscles.
Multiple organ pathologies are divided into several types, which determines the symptoms of the disease:
- With damage to the renal vessels (and this occurs in most patients), an increase in blood pressure is noted. Hypertension is persistent, persistent, causing a severe degree of retinopathy. Loss of visual function is possible. Urinalysis reveals proteinuria (up to 3 g/day), micro or macrohematuria. In some cases, the vessel expanded by the aneurysm ruptures, forming a perirenal hemorrhage. Renal failure develops during the first three years of the disease.
- If the vessels located in the abdominal cavity are damaged, the symptoms appear already at an early stage of periarteritis nodosa. The main symptoms are abdominal diffuse pain, persistent and progressive. Dyspeptic disorders are noted: diarrhea mixed with blood up to ten times a day, emaciation, bouts of nausea and vomiting. If ulcerative perforation occurs, signs of acute peritonitis develop. There is a risk of gastrointestinal bleeding.
- With damage to the coronary vessels, pain in the heart is not typical. There are heart attacks, mostly small-focal character. The phenomena of cardiosclerosis are rapidly increasing, which entails the appearance of arrhythmias, signs of cardiac insufficiency.
- When the respiratory system is affected, bronchospasm, hypereosinophilia, and eosinophilic infiltrates in the lungs are found. The formation of vascular inflammation of the lungs is characteristic: the disease is accompanied by cough, scanty sputum discharge, less often - hemoptysis, aggravated by symptoms of insufficient respiratory function. On the radiograph, a sharply enhanced vascular pattern is visualized in the form of a congestive lung, infiltration of the lung tissue (mainly in the basal region).
- When the peripheral nervous system is involved in the process, asymmetric poly and mononeuritis is noted. The patient is worried about severe pain, numbness, sometimes muscle weakness. The legs are more commonly affected, less often the arms. Some patients develop polymyeloradiculoneuritis, paresis of the feet and hands. Often, peculiar nodules are found along the trunks of blood vessels, ulcers and foci of necrosis of the skin. Possible necrosis of soft tissues and the development of gangrenous complications.
The initial clinical picture of periarteritis nodosa reveals itself as fever, feeling very tired, increased night sweats, loss of appetite and emaciation, muscle weakness (especially felt in the limbs). Many patients develop muscle pain, accompanied by focal ischemic myositis and joint pain. The affected muscles lose their strength, the development of inflammatory processes in the joints is possible.
The severity of the first signs is different, which depends to a greater extent on which organ or organ system is affected:
- defeat of the peripheral nervous system is manifested by motor and sensory disorders of the ulnar, median and peroneal nerves, it is also possible to develop distal symmetrical polyneuropathy;
- the central nervous system responds to the pathology with headaches, strokes (ischemic and hemorrhagic) are less common against the background of high blood pressure;
- kidney damage is manifested by arterial hypertension, a decrease in the daily amount of urine, uremia, general changes in urine sediment, the appearance of blood and protein in the urine in the absence of cellular casts, back pain, and in severe cases - signs of kidney failure;
- the digestive tract makes itself felt with pain in the liver and abdomen, nausea, vomiting, diarrhea, symptoms of malabsorption, intestinal perforation and peritonitis;
- from the side of the heart, there may be no pathological signs, or symptoms of heart failure appear;
- on the skin there is a livedo reticularis, reddening painful nodules, a rash in the form of vesicles or vesicles, areas of necrosis and ulcerative lesions;
- the genitals are affected by the type of orchitis, the testicles become painful.
Kidney damage in nodular periarteritis
The kidneys are affected in more than 60% of patients with periarteritis nodosa. Moreover, in more than 40% of cases, the lesion is a renal failure.
The likelihood of developing kidney disorders depends on the gender and age category of patients, on the presence of pathologies of the skeletal muscles, the valvular system of the heart and the peripheral nervous system, on the type of course and phase of the disease, on the presence of viral hepatitis antigen and cardiovascular values.
The rate of development of nephropathy is directly determined by the level of C-reactive protein and rheumatoid factor in the blood.
Renal disorders in periarteritis nodosa are due to stenosis and the appearance of microaneurysms of the renal vessels. The degree of pathological changes correlates with the severity of disorders of the nervous system. It must be understood that kidney damage dramatically reduces the chances of survival of patients. However, this issue of the influence of certain disorders of renal function on the course of periarteritis nodosa has not been studied enough.
The inflammatory process usually extends to interlobar arterial vessels and less often to arterioles. Presumably, glomerulonephritis is uncharacteristic of periarteritis nodosa and occurs mainly against the background of microscopic angiitis.
The rapid aggravation of renal failure is due to numerous heart attacks in the kidneys.
The picture of the defeat of the cardiovascular apparatus is noted in every second case out of ten. Pathology is manifested by hypertrophic changes in the left ventricle, increased heart rate, and cardiac arrhythmias. Inflammation of the coronary vessels in periarteritis nodosa can provoke the appearance of angina pectoris and the development of myocardial infarction.
In macropreparations, in more than 10% of cases, nodular thickenings of the type of a rosary are found, from a few millimeters to a couple of centimeters in diameter (up to 5.5 cm in case of damage to large vascular trunks). The section shows an aneurysm, often with thrombotic filling. The final diagnostic role is played by histology. A typical feature of periarteritis nodosa is a polymorphic vascular lesion. There is a combination of different types of connective tissue disorganization:
- mucoid swelling, fibrinoid changes with further sclerosis;
- narrowing of the vascular lumen (up to obliteration), the formation of blood clots, aneurysms, in severe cases - rupture of blood vessels.
Vascular changes become a trigger in the development of necrosis, atrophic and sclerotic processes, hemorrhages. Some patients have phlebitis.
In the heart, atrophy of the fatty layer of the epicardium, brown myocardial dystrophy, and in hypertension, left ventricular hypertrophy are found. With coronary lesions, focal myocardial necrosis, dystrophy and atrophy of muscle fibers develop. Myocardial infarctions are relatively rare - mainly due to the formation of collateral blood flow. Thrombosculitis is found in the coronary arterial trunks.
Skin manifestations of periarteritis nodosa
Skin signs of the disease are observed in every second patient with periarteritis nodosa. Often the appearance of rashes becomes the first, or one of the first signs of a violation. Typical symptoms are:
- vesicular and bullous rash;
- vascular papulo-petechial purpura;
- sometimes - the appearance of subcutaneous nodular elements.
In general, the skin manifestations of periarteritis nodosa are heterogeneous and varied. General signs may be:
- the nature of the rash is inflammatory;
- the rash is symmetrical;
- there is a tendency to swelling, necrotic changes and hemorrhages;
- at the initial stage, the rash is localized in the region of the lower extremities;
- evolutionary polymorphism is noted;
- there is a connection with pre-existing infections, medication, temperature changes, allergic processes, autoimmune pathologies, impaired venous circulation.
Skin lesions vary in variety, ranging from macules, nodules, and purpura to necrosis, ulcers, and erosions.
Nodular periarteritis in children
Juvenile polyarteritis is a form of polyarteritis nodosa that is found predominantly in pediatric patients. This variant of the course of the disease is distinguished by a hyperergic component, most of the peripheral vessels are damaged, there is a considerable risk of developing thromboangiitis complications in the form of dry tissue necrosis, gangrenous processes. Visceral disorders are relatively weak and do not affect the outcome of the pathology, but there is a tendency to a long course with periodic relapses.
The classic form of juvenile polyarteritis has a severe course: kidney damage, high blood pressure, abdominal ischemia, cerebral vascular crises, inflammation of the coronary vessels, pulmonary vasculitis, and numerous mononeuritis are noted.
Among the causes of the disease are considered mainly allergic and infectious factors. The classic form of periarteritis nodosa is associated with a viral infection of hepatitis B. Often the onset of the disease is noted along with acute respiratory viral infections, otitis and tonsillitis, somewhat less often with the introduction of vaccines or drug therapy. A genetic predisposition is not excluded either: often, rheumatological, allergic or vascular pathologies are found in the direct relatives of a sick child.
The incidence of periarteritis nodosa in childhood is unknown: the disease is diagnosed very rarely.
Pathogenesis is often due to immunocomplex processes with an increase in complement activity and leukocyte accumulation in the area of fixation of immunocomplexes. An inflammatory reaction occurs in the walls of small-caliber and medium-caliber arterial trunks. As a result, proliferative-destructive vasculitis develops, the vascular bed is deformed, blood circulation is inhibited, the rheological and coagulation properties of blood are disturbed, thrombosis and tissue ischemia are noted. Gradually, wall fibrosis is formed, aneurysms with a diametrical size of up to 10 mm are formed.