Cryoglobulinemic vasculitis is a severe pathology occurring against the background of hemorrhagic rash, renal failure and Raynaud's syndrome. According to statistics, cryoglobulins in the blood are found in 40% of the world's population, although they do not necessarily provoke pathological processes in the body.
The disease is characterized by the deposition of cryoglobulins on the walls of vessels with a small diameter. Cryoglobulins are also found in the blood itself and dissolve when heated.
Epidemiology
Cryoglobulinemic vasculitis is classified under ICD-10 category D89.1 under the name Cryoglobulinemia.
There are no statistics on the incidence of vasculitis itself. However, 40% of all inhabitants of our planet have cryoglobulins in their 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, this is the number of people who have symptoms characteristic of vasculitis.
The disease can appear 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 occurs more often in women, approximately 1.3-1.5 times. The likelihood of developing the disease increases as the body ages.
Symptomatics
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 severe 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 small vessels being affected.
Most often the rash appears on the lower extremities. In addition to the fact that it is clearly visible, it is also perfectly palpable. After the rash disappears, the skin does not become clear, brown spots remain - these are the remnants of red blood cells.
In some patients, cryoglobulinemic vasculitis does not end with the rash disappearing, but necrosis begins, ulcers appear. Vivid signs of circulatory disorders may appear on the fingertips, nose and ears, such manifestations are called Raynaud's syndrome. This condition is dangerous because there is a high risk of developing gangrene.
Almost always, patients notice joint pain, and not only large, but also small joints hurt. Exacerbation usually begins after hypothermia. Can developarthritis.
It is not uncommon for internal organs to be involved in the inflammatory process. If it is lungs, then shortness of breath and cough may appear. Cryoglobulinemic vasculitis is the most severe, if the kidneys are involved in the process, a severe form of glomerulonephritis 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 appear.
Etiology
Vasculitis can be 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 cause of 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 vasculitis. Other infections or collagenosis can provoke the development of the disease.
Diagnosis
Most often, the primary diagnosis can be made during the 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, if vasculitis is suspected, 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 thereIf there are structural changes in the internal organs, doctors may prescribe an ultrasound, MRI, x-ray.
Treatment
Cryoglobulinemic vasculitis can be treated in a variety of ways, depending on the symptoms and nature of origin. Plasmaphoresis 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 proceeds in an acute form, then drugs are prescribed that depress 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 length and quality of life of the patient.
If gangrene of the extremities has begun, surgery may be recommended.
Course of disease
To date, there is no generally accepted system for assessing the severity of this disease. However, there are several states:
- Life-threatening patient. This group includes patients who have large ulcers, rapidly progressing glomerulonephritis, the disease has affected the central nervous system, there is necrosis of the fingertips.
- Heavy form. Patients present with nephrotic syndrome, skin lesionsnon-ulcer character.
- Moderate degree. It is characterized by palpable purpura, myalgia, and mild polyneuropathy.
After the patient enters a period of remission, he must be prescribed a combination antiviral therapy, which lasts from 2 to 4 months.
Evaluation of treatment effectiveness
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 are 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 fairly serious disease that requires serious treatment. Naturally, drugs have many side effects. In particular, commonly used glucocorticoids and "Cykylophosmamide" can cause hepatitis C to become chronic.
Glucocorticoids in high doses can provoke vascular catastrophes, for example, the same thrombosis. And multiple transfusions of donor plasma can provoke other infections in the body.
However, it should be remembered that this type of vasculitis, if untreated, has a poor prognosis, there are no preventive measures to avoid such a pathology in the future.
