What is Hemorrhagic Vasculitis?
Hemorrhagic vasculitis (Schonlein-Henoch disease) is a typical representative of small vessel vasculitis. The disease is not uncommon, and more often in children.
Pathogenesis during Hemorrhagic Vasculitis
In the development of the disease, the main importance is attached to immunopathological mechanisms, and drugs, allergenic components of food, bacterial or viral products and even tumor antigens can act as triggering antigens. In many cases, it is possible to establish the presence of IgA-containing immune complexes in the blood serum and in the foci of skin and renal lesions. There are indications of the participation of both classical and alternative pathways of complement activation. Cases of a combination of hemorrhagic vasculitis with congenital absence of the second component of complement are described. The total activity of complement in the serum of most patients is normal.
Histological examination reveals only small vessel vasculitis – arterioles and capillaries. An important feature – the complete absence of lesions of the muscular arteries. Characterized by neutrophilic infiltrates, a moderate number of mononuclears, an abundance of disintegrating leukocytes (hence the descriptive term – “leukocytoclastic vasculitis”), fibrinoid changes, hemorrhages. Perhaps the complete reverse development of these tissue changes. In contrast to classical polyarteritis, all altered vessels with hemorrhagic vasculitis have similar signs of damage at the time of the study, that is, they are at the same morphological stage of the disease.
Symptoms of Hemorrhagic Vasculitis
The disease usually begins in spring, and in many patients after an infection of the upper respiratory tract. The main clinical sign of the disease is an abundant hemorrhagic rash with a diameter of, as a rule, 3-10 mm, localized mainly on the legs (especially the legs and feet) and buttocks. In many patients it protrudes slightly over the skin, i.e. it has a papular-hemorrhagic character, and occasionally is accompanied by weak itching, as well as a slight swelling of the feet, legs or periorbital. Often the appearance of the rash is preceded by a tingling sensation or a slight itch in the places of its future localization. A hemorrhagic rash on the upper half of the body and arms occurs much less frequently and is less pronounced, but quite possible. Damage to the skin of the neck and face is completely atypical. Hemorrhages on the legs can be very abundant and often coalesce. In the absence of new eruptions, after 2–3 days they begin to fade and subsequently disappear without a trace. The rash usually appears suddenly; Subsequently (especially after long standing or walking), new “waves” of eruptions are often followed, which may be accompanied by a moderate increase in temperature, some increase in ESR and neutrophilia by leukocytosis. Platelet count is normal.
In many patients, the clinical signs of the disease are exhausted by skin manifestations. However, other syndromes, often articular, often come to the fore. It manifests itself in reversible, non-migratory, arthritis with damage to usually more than one joint. The most characteristic involvement in the process of the leg joints is the ankle and knee; less common is the involvement of the wrist and elbow. A number of patients have only arthralgia. Synovial fluid from the affected joints has a distinctly inflammatory character with high neutrophilic leukocytosis. Arthritis always ends in complete recovery without any elements of destruction. Involvement of the kidney in the process occurs in a few patients and is usually mild and transient. It is manifested by hematuria and small proteinuria. Much less frequently, kidney damage progresses and leads to kidney failure, which can cause death. Histological detection of focal glomerulitis with endothelium proliferation and fibrinoid deposits. In some patients, the vessels of the digestive tract are affected, which is manifested by abdominal pain, sometimes colicky. The causes of it are usually hemorrhages and edema of the intestinal wall or mesentery. In rare cases, there is diarrhea with protein loss or intestinal bleeding; Inversion of intestines and perforations are described. It is significant that in a number of patients with hemorrhagic vasculitis, abdominal pain syndrome is the first sign of the disease, causing serious diagnostic difficulties and sometimes leading to unnecessary surgical interventions. In the literature there are also isolated descriptions of hemorrhages in the eye tissue and in the central nervous system with severe headache, hypertensive encephalopathy, and seizures.
In most cases, the disease is mild and ends with recovery, although it often recurs. The prognosis becomes serious with the evolution of renal changes in chronic glomerulonephritis and with such very rare manifestations as intestinal bleeding, torsion or perforation of the intestines and hemorrhage in the brain.
Treatment of Hemorrhagic Vasculitis
Treatment of patients with hemorrhagic vasculitis is a difficult task; There are no drugs that effectively suppress the main pathological process, regardless of its location. It is necessary to exclude the impact of deliberately active antigenic effects, especially those that chronologically coincided with the clinical manifestations of the disease. In the active phase of the disease, contrasting temperature effects, prolonged standing and long walking should be avoided, as this may significantly increase skin hemorrhages (especially on the legs). The use of corticosteroids (starting with 20-40 mg of prednisolone per day) is considered most effective for severe arthritis and, according to some reports, for gastrointestinal bleeding caused by hemorrhagic vasculitis. The effect on skin rashes and kidney damage is less distinct, although in a number of patients the corresponding clinical and laboratory manifestations may slightly decrease under the influence of hormonal therapy.
We observed a positive effect in various sites of the disease (especially in the predominantly dermal variant) of prodectin, administered at 0.75-1.5 g / day for 2-3 months. Prodectin can be combined with any drugs used in the treatment of hemorrhagic vasculitis. Since arthritis in this disease is usually mild, we prefer to use NSAIDs, primarily voltarenum (150-100 mg / day) for treatment as first-line drugs. In the case of a relapsing course of the disease, long-term administration of plaquenil at a dose of 0.2 g per day or delagil (at 0.25 g) should be included in the treatment complex. In case of persistent and especially progressive kidney damage, it is advisable to use immunosuppressants such as azathioprine instead of delagil and plaquenil. In such cases, a positive therapeutic effect of plasmapheresis was also noted.