Treatment of Systemic Vasculitis

Vasculitides — are a group of autoimmune diseases that are characterized by inflammation of blood vessels. As a result, blood circulation and the work of different organs may be impaired. Vasculitides often manifest as pain in the joints and muscles, weakness, skin rashes, and other symptoms that depend on the localization and prevalence of the inflammatory process.

Systemic vasculitides may have different courses: in some patients, skin manifestations and joint involvement predominate, while in others — symptoms from the lungs, kidneys, nervous system, or heart. That is why timely diagnosis is of great importance, because early initiation of treatment helps reduce the risk of internal organ involvement and the development of complications.

At the medical center «Oxford Medical», patients can undergo a consultation with a rheumatologist, the necessary examinations, and receive an individual treatment plan. The doctor will assess the symptoms, test results, and select therapy tactics taking into account the form of the disease and its activity.

Systemic vasculitides unite a large group of diseases that differ in the causes of development, course, and in which vessels and organs are predominantly affected. According to the data of the international classification Chapel Hill Consensus Conference, more than 20 forms of vasculitis have been described, which can affect vessels of different caliber and have different clinical manifestations. However, some types of the disease occur significantly more often than others.

The most well-known types of vasculitis include:

• hemorrhagic vasculitis (IgA vasculitis) — more often affects the small vessels of the skin, joints, intestine and kidneys;

• Behcet's disease — is accompanied by inflammation of vessels of different caliber, ulcers of the mucous membranes, involvement of the eyes, skin and joints;

• polyarteritis nodosa — affects predominantly medium-caliber arteries and can affect the functioning of the kidneys, nervous system, skin and digestive organs;

• giant cell arteritis — inflammation of large arteries, most often in the area of the head and neck, which can cause severe headache and visual impairment;

• granulomatosis with polyangiitis — often affects the respiratory tract, lungs and kidneys;

• microscopic polyangiitis — a disease of small vessels that most often affects the kidneys and lungs;

• eosinophilic granulomatosis with polyangiitis — combines signs of vasculitis with bronchial asthma and an increased level of eosinophils in the blood;

• urticarial vasculitis — manifests as rashes similar to urticaria, which arise due to inflammation of the small vessels of the skin.

Depending on the form of the disease, skin manifestations, joint pain, symptoms from the lungs, kidneys, nervous system or other organs may predominate. That is why, to establish an accurate diagnosis, it is important to determine not only the presence of vasculitis, but also its specific type.

The exact cause of the development of many forms of vasculitis remains unknown. It is believed that the disease is associated with impaired functioning of the immune system, due to which inflammation of the walls of blood vessels occurs.

Vasculitis can be primary, when it occurs as an independent autoimmune disease, or secondary, when it develops against the background of another disease, infection or under the influence of certain medicinal products.

Factors that may be associated with the development of vasculitis include:

• viral and bacterial infections;

• other autoimmune diseases;

• some medicinal products;

• hereditary predisposition to disorders of the functioning of the immune system;

• long-lasting inflammatory processes in the body.

In most cases, vasculitis is considered an autoimmune inflammatory process in which the immune system mistakenly attacks the body's own tissues. Secondary vasculitis can develop against the background of other diseases. In particular, it can be detected in patients who have rheumatoid arthritis, systemic lupus erythematosus, chronic infections or other conditions accompanied by long-lasting inflammation.

Skin manifestations can be different. Skin vasculitis can manifest as small hemorrhages, red spots, painful nodules or ulcers. For certain forms of the disease, an urticarial rash is characteristic, which externally resembles urticaria, but is associated not with allergy, but with inflammation of the small vessels of the skin.

If the inflammatory process affects the lungs, shortness of breath, chest pain or a dry cough that does not go away for a long time may occur. When the kidneys are affected, swelling sometimes appears, blood pressure increases or changes are detected in urine tests.

In some patients, the disease affects the nervous system, which can manifest as numbness of the limbs, tingling, decreased sensitivity or muscle weakness. That is why the symptoms of vasculitis can differ significantly in different people.

Doctors of «Oxford Medical» recommend consulting a rheumatologist if skin rashes are combined with joint pain, prolonged temperature, weakness or other unclear symptoms. Early establishment of the diagnosis helps to start treatment faster and reduce the risk of damage to internal organs.

Diagnosis of systemic vasculitides begins with a consultation with a rheumatologist. The doctor clarifies complaints, duration of symptoms, presence of rashes, joint pain, shortness of breath, cough, changes from the kidneys and other manifestations that may indicate inflammation of the vessels.

To clarify the diagnosis, the following may be prescribed:

• general blood and urine tests;

• inflammation markers (ESR, C-reactive protein);

• biochemical tests to assess kidney and liver function;

• immunological studies, in particular determination of autoantibodies;

• X-ray or computed tomography of the chest organs according to indications;

• ultrasound examinations of internal organs;

• MRI of vessels, if it is necessary to assess the condition of the vascular wall and blood flow in more detail;

• biopsy of the affected tissue, if it is necessary to confirm the nature of the inflammation.

Systemic vasculitides can manifest in different ways, so one test or one examination is usually not enough to establish the diagnosis. The doctor evaluates the combination of symptoms, laboratory indicators and results of instrumental diagnostics.

According to National Health Service (NHS), treatment of systemic vasculitides is selected individually and depends on the type of disease, its activity and the presence of damage to internal organs. The main goal of therapy is to reduce inflammation in the vessels, prevent their further damage and reduce the risk of complications from the kidneys, lungs, heart, nervous system or skin.

Treatment may include:

• glucocorticosteroids — for rapid suppression of active inflammation;

• immunosuppressive drugs — for control of excessive immune reaction and prevention of exacerbations;

• targeted drugs — for certain forms of vasculitis, if standard therapy is insufficient;

• drugs for control of arterial pressure, blood clotting, anemia or other concomitant disorders;

• treatment of infections or other diseases, if vasculitis has a secondary character.

Doctors of «Oxford Medical» recommend not stopping therapy independently after symptoms decrease. With systemic vasculitides, external improvement does not always mean complete fading of inflammation, therefore control tests and correction of treatment according to examination results are important.

Complications of vasculitis depend on the form of the disease, the activity of inflammation and the organs that are involved in the pathological process. Due to damage to the vessels, the blood supply to tissues may be impaired, which affects their work and the functioning of the body as a whole.

Possible complications of vasculitis:

• kidney damage with gradual decrease in their function;

• inflammatory changes in the lungs with breathing impairment;

• damage to the nervous system with development of sensitivity or movement disorders;

• narrowing or damage to blood vessels;

• formation of blood clots and circulatory disorders;

• deep vein thrombosis and other vascular complications.

With certain forms of the disease, irreversible changes from the kidneys, lungs or nervous system may occur. That is why timely diagnosis and correctly selected treatment are important for prevention of complications and preservation of the patient’s quality of life.

For most primary vasculitides, there is no method that would completely prevent the development of the disease. At the same time, timely detection of symptoms, control of concomitant diseases and adherence to the doctor’s recommendations can help reduce the risk of exacerbations and complications.

For prevention of the progression of vasculitis, it is recommended:

• not to stop the prescribed treatment without agreement with the doctor;

• to treat infectious diseases in a timely manner;

• to control arterial pressure;

• to give up smoking;

• to adhere to a balanced diet and maintain sufficient physical activity according to the state of health.

Regular follow-up with a doctor after diagnosis is especially important. Even if symptoms have temporarily disappeared, the inflammatory process may persist or resume over time. That is why monitoring tests and following the doctor’s recommendations are an important part of preventing complications and maintaining a stable course of the disease.

The cost of treatment of systemic vasculitides depends on the form of the disease, the necessary scope of diagnostics and the selected therapy tactics. In some cases, a consultation with a rheumatologist and basic examinations are sufficient, in others additional tests, instrumental diagnostics and other medical services may be needed.

The doctor will guide you in more detail regarding the necessary examinations, treatment tactics and its cost during the consultation. You can find out the prices for doctors’ consultations here. To make an appointment at a time convenient for you, contact the contact center or use the online appointment form on the website.

The information is for informational purposes and does not replace a doctor’s consultation.

Sources:

• Chapel Hill Consensus Conference
• National Health Service
• European Alliance of Associations for Rheumatology