What is vasculitis?
Vasculitis refers to a group of diseases that occur as a result of inflammation (inflammation) of blood vessels, resulting in complaints related to malnutrition in tissues such as lungs, kidneys, nerves and skin fed by that vessel. Sometimes it can have life-threatening consequences. There are many types of vasculitis, and most are rare. Both arteries (arteries) and veins (veins) can be involved. Therefore, it causes a wide variety of findings. Some of them are:
– Numbness and weakness in the hand or foot,
– Shortness of breath and cough,
– Skin rashes- raised purpura (small red dot rash), nodule ( swelling), or ulcerated sores.
– Newly developed severe hypertension
– Hypertension with involvement of the kidneys, edema
– May cause complaints such as painful redness in the eye, temporary or permanent vision loss.
What is the cause of vasculitis?
There is a group of diseases under the title of vasculitis, and the cause of most of them is unknown. In addition to genetics, it is thought that many environmental factors that affect the immune system of the person, especially infections and smoking, contribute to the development of vasculitis. With the person’s immune (immune) system, attacking his own blood vessels, accompanied by signs of inflammation; it may narrow in the vessel, causing obstruction or tearing due to damage to its wall. With the deterioration of the blood supply of the tissue supplied by the affected vessels, various complaints develop due to the insufficiency of that tissue. Small vessel vasculitis may develop in all autoimmune systemic connective tissue diseases (such as lupus, rheumatoid arthritis, Sjögren’s syndrome). Vasculitis may accompany hepatitis B and C infections.
Who gets vasculitis?
Vasculitis affects people of both sexes and all age groups. Some forms of vasculitis, such as Kawasaki disease, develop only in children. Henoch-Schönlein purpura is more common in children than adults. Giant cell arteritis is seen over 50 years of age. There are many types of vasculitis and most of them are rare diseases.
How is vasculitis diagnosed?
The patient’s complaints and abnormalities in examination and test results may suggest vasculitis to the doctor. Sometimes further imaging tests (such as angiography) or biopsy of the affected vessel or organs may be required to confirm the diagnosis.
Vasulites are generally classified according to the vessel diameter they involve.
Large vessel vasculitides: Involves the aorta (the main artery leaving the heart) and its main branches. This group includes: Giant cell arteritis (Temporal arteritis), Takayasu arteritis, aortitis due to Cogan’s syndrome, aortitis in spondyloarthropathies and isolated aortitis.
Medium-sized vessel vasculitides: Medium-sized vessels are the vessels that go to more organs in our body and are called by their names (such as hepatic artery, branching points of the renal artery, mesentery artery, coronary artery). This group includes Kawasaki disease and Polyarteritis nodosa.
Small vessel vasculitides: ANCA (anti-neutrophil cytoplasmic antibody)-related vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg-Straus syndrome), lupus, Sjögren’s syndrome or rheumatoid arthritis-associated vasculitides, cryoglobulin syndrome Goodpasture vasculitis , drug-induced vasculitides, hepatitis B or C-associated vasculitides, malignancy-associated vasculitides (such as multiple myeloma, hairy cell leukemia)
Other: Primary angiitis of the central nervous system; It holds small and medium sized vessels.
Behçet’s disease and relapsing polychondritis can involve arteries and veins of all sizes.
How is vasculitis treated?
Glucocorticoids: Commonly known as steroids or cortisone. It is used in different doses depending on the type and severity of vasculitis in order to suppress disease symptoms and inflammation in the early period (active) of vasculitis. In cooperation with your doctor, you can get information about the side effects of steroids and learn about prevention methods.
Other drugs: These are drugs that reduce the need for steroids. Most are immunosuppressant treatments. Cyclophosphamide is the strongest of them all. It is preferred in case of vital organ involvement. Methotrexate and azathioprine are other drugs. One of the biological treatments, rituximab has been used in the treatment of vasculitis resistant to other treatments in recent years. Plasmapheresis and intravenous immune globulin therapy are other treatment options used in some resistant vasculitis cases.
Surgery: Rarely needed. It may be required in cases such as grafting of vascular tissue that has been severely damaged due to severe vasculitis or organ transplantation.
Recommendations for patients with vasculitis:
Vasculitis can be short-term or lifelong. Preventing damage to vital organs and preventing future damage is the basis of treatment. Your treatment is planned by taking into account the benefits and harms (side effects) of the drugs you usually use. By being informed about the side effects of your medications, you can take precautions for preventable side effects. For example, side effects such as steroid-induced osteoporosis, Cushing’s syndrome, high blood sugar and blood pressure; You can take precautions such as a steroid diet, calcium and vitamin D replacement, muscle-strengthening exercises, blood sugar and blood pressure monitoring. To prevent infections, you can get the flu and pneumonia vaccine.
