Cryoglobulinemic vasculitis is a severe pathology occurring against the background of hemorrhagic rash, renal failure and Raynaud's syndrome. According to statistics, cryoglobulins are found in blood in 40% of the world's population, although it does not necessarily provoke pathological processes in the body.
The disease is characterized by the deposition of cryoglobulins on the walls of small diameter vessels. Cryoglobulins are also found in the blood itself and dissolve during heating.
Epidemiology
ICD-10 cryoglobulinemic vasculitis is assigned to category D89.1 under the name Cryoglobulinemia.
Statistics on the incidence of vasculitis itself do not exist. However, in 40% of all the inhabitants of our planet, cryoglobulins are present in blood serum.
There are no statistics on the incidence of vasculitis among patients with hepatitis C. But it is assumed that we are talking about 11-23% of people with hepatitis, in any case, it is in such a number of people that symptoms characteristic of vasculitis occur.
The disease can occur at any age, both in a 20-year-old person and in a 70-year-old. However, it has already been established that the disease is more common in women, about 1.3-1.5 times. The likelihood of developing the disease increases with the aging of the body.
Symptomatology
Pathology is characterized by a fairly wide list of symptoms. But the most characteristic manifestation of the disease includes skin manifestations, which are also called the Meltzer triad. Secondary symptoms include pronounced weakness and joint pain.
At the initial stage, the skin becomes less sensitive, then a rash appears in the form of purple. These are small and multiple hemorrhages on the surface of the skin. The rash is caused by the small vessels being affected.
Most often, the rash appears on the lower extremities. Besides the fact that it is clearly visible, it is also perfectly palpable. After the rash disappears, the skin does not become clean, brown spots remain - these are the remains of red blood cells.
In some patients, cryoglobulinemic vasculitis does not end with a rash, but necrosis begins, ulcers appear. Vivid signs of a blood supply disturbance may appear on the tips of the fingers, nose and ears, such manifestations are called Raynaud's syndrome. This condition is dangerous because there is a big risk of developing gangrene.
Almost always, patients note joint pain in themselves, and not only large, but also small joints hurt. Exacerbation usually begins after hypothermia. Arthritis may develop.
There are frequent cases when the internal organs are involved in the inflammatory process. If it is light, shortness of breath and coughing may appear. The most severe cryoglobulinemic vasculitis occurs if the kidneys are involved in the process, a severe form of glomerulonephritis may begin, or an acute form of renal failure may begin.
Peripheral nerves may be affected. If the vessels of the brain are affected, motor paralysis or speech problems may occur.
Etiology
Vasculitis can act as an independent primary disease, and it is called essential cryoglobulinemic vasculitis. But this form is very rare, and most often it is not possible to establish the reason for its appearance. This form is observed in only 10% of all patients.
The most common secondary form is cryoglobulinemic vasculitis in hepatitis C, although often this pathology is not the only provocateur of the development of vasculitis. Other infections or collagenosis can provoke the development of the disease.
Diagnostics
Most often, the initial diagnosis can be made upon examination of the patient. To confirm it, laboratory immunological studies are carried out. In particular, the amount of cryoglobulins in the blood is determined. Without fail, with suspected vasculitis, an analysis is carried out for the presence of hepatitis C, blood is checked for the presence of other bacterial infections and viruses.
To find out if there are structural changes in the internal organs, doctors can prescribe an ultrasound, MRI, x-ray.
Treatment
Cryoglobulinemic vasculitis can be treated with a variety of methods, depending on the symptoms and nature of the origin. Plasmophoresis can be used to purify the blood.
To reduce the inflammatory process on the walls of blood vessels, glucocorticoid hormones are prescribed.
If the disease is protracted and occurs in an acute form, then drugs are prescribed that inhibit the immune system - cytostatics. Naturally, in the presence of hepatitis C, first of all, the primary disease is treated.
However, it should be understood that cryoglobulinemic vasculitis is an incurable disease. However, modern treatment methods can suppress its development and reduce the risk of exacerbations. The main goal of treatment is to increase the duration and quality of life of the patient.
If gangrene of the extremities has begun, surgery may be recommended.
Course of the disease
To date, there is no generally accepted system for assessing the severity of this disease. However, there are several conditions:
- Life threatening patient. This group includes patients who have large ulcers, glomerulonephritis progresses rapidly, the disease affects the central nervous system, and there is necrosis of the fingertips.
- Severe form. Patients present with nephrotic syndrome, non-ulcerous skin lesions.
- Moderate degree. It is characterized by palpable purpura, myalgia, polyneuropathy is weakly expressed.
After the patient begins a period of remission, he is definitely prescribed combination antiviral therapy, which lasts from 2 to 4 months.
Evaluation of the effectiveness of treatment
The doctor evaluates the effectiveness of the prescribed course of therapy according to the general condition of the patient. Acute phase indicators are checked: CRP and ESR. Clinical manifestations are assessed whether there is a decrease in symptoms. The kidneys are checked, in particular, the concentration of creatinine and GFR is specified.
Virological remission in the presence of hepatitis C is checked by determining the presence or absence of virus DNA in the blood serum.
Possible complications
Cryoglobulinemic vasculitis (ICD-10 D89.1) is a rather serious disease that requires serious treatment. Naturally, drugs have many side effects. In particular, the commonly used glucocorticoids and Cyclophosmamide can cause hepatitis C to become chronic.
Glucocorticoids in large doses can cause vascular accidents, for example, the same thrombosis. And multiple transfusions of donor plasma can provoke the appearance of other infections in the body.
However, it should be remembered that this type of vasculitis in the absence of treatment has an unfavorable prognosis, there are no preventive measures to avoid such a pathology in the future.