What is Vasculitis?
The term “vasculitis” includes a group of autoimmune diseases, common feature of condition is inflammation of blood vessels. However, symptoms and course of these diseases can show great differences.
Primary and secondary vasculitis must be differentiated: Primary vasculitis is an autoimmune disease independent of the group of inflammatory rheumatic diseases. The major forms of vasculitis are classified into three major groups depending on whether vascular inflammation affects small, medium or large blood vessels:
Vasculitis of large blood vessels
- Temporal arteritis (arteritis temporalis)
- Takayasu’s arteritis
Vasculitis of medium blood vessels
- Polyarteritis nodosa
- Kawasaki Disease (Morbus Kawasaki)
Vasculitis of small blood vessels
- Churg-Strauss Syndrome
- Microscopic panarteritis (microscopic polyarteritis)
- Henoch-Schöenlein Purple
- Essential cryoglobulinemia vasculitis
- Cutaneous leukocytoclastic vasculitis
Secondary vasculitis is caused by medications, infections or other autoimmune diseases. The most common form of vasculitis is the arteritis of the temporal, an inflammation of the temporal artery. With an incidence that varies according to the different countries, it ranges from 0.43-23.3 per 100,000 inhabitants according to the published rates. This pathology affects women three times more than men.
Vasculitis is an inflammatory disease of the blood vessels (from the Latin vasculum = vasos and itis = inflammation) which are caused by reactions of the immune system. By definition, term vasculitis includes several autoimmune diseases.
The different forms of vasculitis occur with different incidences: the most common form (temporal arteritis) varies according to the different countries according to published rates, which ranges ranges from 0.43-23.3 per 100,000 inhabitants. Women are affected three times more than men. Secondary vasculitis is also very common. All other forms of vasculitis are rarer with fewer than 1,000 new cases per year in Europe.
What is Primary Vasculitis?
Primary vasculitis is an independent autoimmune disease. It forms a group of different special vasculitis including, for example:
Temporal Arteritis: A primary vasculitis is condition which medial and large blood vessels usually swell in the upper half of the body and especially the head. This form of vasculitis affects mostly old people who are older then 60.
Temporal arteritis is closely related to polymyalgia (polymyalgia rheumatica) that may appear simultaneously. If those affected take cortisone (higher doses initially and small doses throughout life, symptoms improve rapidly.
Takayasu’s Arteritis:This type of primary vasculitis is similiar to temporal arteritis which large arteries are inflamed. Aorta and its branches are especially inflamed. This form of vasculitis mainly affects young people under 40 years.
Poliarteritis Nodosa: A particularly serious primary vasculitis is poliarteritis nodosa. It is characterized by inflammation of small and medium blood vessels and requires long-term treatment with drugs that suppress the immune system (immunosuppressive drugs).
At the same time, some people with this form of vasculitis have hepatitis due to hepatitis B virus which is probably associated with the existence of poliarteritis nodosa.
Wegener’s Granulomatosis: A primary vasculitis with inflammation at the granulomatosis of small and medium vessels is called Wegener’s granulomatosis. Inflammation of the vessels mainly affects the respiratory tract (nose, sinuses, trachea and lungs) in the locoregional phase and later, it can also affect other organs such as eye or kidneys.
Churg-Strauss Syndrome: Another primary vasculitis with inflammation of nodes (granulomatosis) of small and medium blood vessels is Churg-Strauss vasculitis. Like Wegener’s disease, this type also usually affects the respiratory tract. Churg-Strauss vasculitis is always accompanied by bronchial asthma. Organs such as the nerves of the body and heart (endomyocarditis) are usually involved.
What is Secondary Vasculitis?
A secondary vasculitis occurs in the context of rheumatic diseases, tumor diseases or can be caused by medications or infections. It usually affects small vessels. A typical secondary vasculitis is vasculitis of small vessels of the skin in people who have severe rheumatoid arthritis.
Another secondary vasculitis is hypersensitivity vasculitis: this form is due to infections or medications. Unlike vasculitis secondary to rheumatoid arthritis, hypersensitivity vasculitis usually has a good prognosis.
Behcet’s Disease: Vasculitis of the small and medium vessels which occur mainly in the eastern Mediterranean and Japan is called Morbus Behcet. Causes of this form of vasculitis are unknown, it can not be classified into the two main groups (primary and secondary vasculitis). Behcet’s disease may be benign, but there may be life threatening evolution when inflammation of the vessels of the heart, lungs or central nervous system (CNS: brain and spinal cord).
Primary vasculitis is a disease of the autoimmune, independent, inflammatory and the causes of which are unknown. On the contrary, possible causes of the secondary vasculitis are:
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Viral infections (hepatitis, HIV)
- Malignant tumors
- Blood and lymphatic diseases
Among the causes of the appearance of a vasculitis play a crucial role the immune system and disorders of its function:
Some forms of vasculitis are responsible for autoantibodies that act against body structures. Especially anti-cytoplasmic antibodies (ANCA) that act against certain white blood cells are important in the onset of a vasculitis. They produce inflammation of medium-sized vessels (eg, Wegener’s granulomatosis, Churg-Strauss syndrome).
In other forms of vasculitis are caused by complexes the body’s own antibodies connect the drugs or components of bacteria and they form immune complexes that are deposited on the walls of blood vessels and damage them. For example, it is common to find a relationship with hepatitis virus in some vasculitis.
Secondarily to the activation of antibodies, certain protein structures of the immune system (this is called complementary system) cause an inflammation of the affected vessels. Depending on the size of affected vessels and the type of vasculitis, different reactions occur.
Vasculitis is initially manifested with symptoms of general illnesses. They often occur before the effects of vasculitis on the affected organs are visible. These general symptoms are:
- Night sweats
- Pain in the joints
- Strong feeling of illness with reduced performance
Subsequent symptoms may appear in specific organs. These symptoms are sometimes very different or indicative of certain vasculitis depending on the form of vasculitis present:
Skin: Redness of the skin with spots (purple), redness in the form of a net (livedo reticularis), places of the skin exposed in the form of ulcerations.
Eyes: Inflammation of the sclera (scleritis) and other structures of the eye with redness of the eye, vision disorders due to infection of the vessels of the retina.
Nervous system: Stroke (vascular disorders of the brain), spasms, depressions, headaches, inflammation of the nerves (mono- and polyneuritis) with pain and paralysis.
Lungs: Cough with blood (hemoptysis) from inflammation of lung tissue.
Otorhinolaryngology: Mucosal disorders (aphthosis), nose bleeds due to inflammation of the nose and sinuses, sudden deafness, rotational vertigo.
Kidneys: Inflammation of renal corpuscles (glomerulonephritis) with blood (microhematuria) and protein in the urine (proteinuria), possible hypertension or loss of function of the kidneys
Heart: Chest pain and sometimes heart failure due to inflammation of the heart muscles and pericarditis and narrowed heart arteries.
Primary Vasculitis Symptoms
The symptoms that occur in a vasculitis can be characteristic of the primary vasculitis:
Temporal Arteritis: The typical symptoms of temporal arteritis in which blood vessels usually swell mainly in the upper half of the body especially in the head. These are strong feeling of general discomforts with elevated erythrocyte sedimentation rate, often muscular pain and headache.
A vascular obstruction in the primary vasculitis can lead to blindness and stroke. Usually the symptoms of temporal arteritis improve rapidly with cortisone.
Takayasu’s Arteritis: The first symptom of Takayasu’s arteritis is usually the lack of pulse in one arm due to the closure by inflammation of blood vessels. This form of vasculitis can also cause joint pain and general symptoms.
Poliarteritis Nodosa: The main symptom of panarteritis nodosa is a strong feeling of general discomfort with muscle pain and testicular pain.
As a result of organic involvement, there are symptoms such as circulation problems in intestines and brain, and renal inflammation. This form of vasculitis leads to the formation of (aneurysms) characteristic nodules, sometimes palpable on the skin and visible by X-rays (angiography).
Wegener’s Granulomatosis: This is a primary vasculitis with nodular (granulomatous) inflammation of the small vessels. Symptoms are related to affected airways by vascular inflammation: nose, sinuses, trachea and lungs. Subsequently vasculitis usually also develops in other organs (eyes and kidneys).
Churg-Strauss Syndrome: Primary vasculitis with nodular (granulomatous) inflammation of small blood vessels occur not only in Wegener’s vasculitis. Churg-Strauss syndrome can also cause nodular inflammation of small blood vessels. Other symptoms of this type of vasculitis are certain white blood cells, which become inflamed both in the blood and in the tissues. Eosinophilic granulocytes also cause normal bronchial asthma.
Secondary Vasculitis Symptoms
Medications, infections or other diseases cause vasculitis. This is called secondary vasculitis symptoms. A typical form of secondary vasculitis is small vessel vasculitis of the skin which can occur in people with severe rheumatoid arthritis. The symptoms are large ulcerations and lesions on the skin of the lower extremities.
A characteristic symptom of hypersensitivity vasculitis is usually redness of the skin with small blemishes which are caused by hemorrhaging in the skin (purple) of the lower extremities, and more rarely in the organs.
The typical symptoms of the form of vasculitis called Behcet’s disease are recurrent ulcers of the mouth (canker sores) and often on the genitals, as well as ocular inflammation (uveitis), joint inflammation and vasculitis in various organs. Infection in the eyes can often lead to blindness. Another disturbance that arises in Behcet’s disease is an inflammation of the veins.
How is Vasculitis Diagnosis Made?
For the diagnosis of vasculitis, there are some initial test that are necessary to be made.
Lab Tests: Several laboratory tests may play a role in the diagnosis of a vasculitis: on the one hand, inflammation of the vessels increases the inflammation values such as sedimentation rate, CRP and white blood cells.
The diagnosis of vasculitis is especially important when viewing a sample of tissue under a microscope (histology), especially biopsy of the skin and the kidneys.
Other organs such as the lungs or all blood vessels such as the temporal artery allow to diagnose the various forms of vasculitis by detecting tissue histological modifications.
Analysis of capillaries by X-rays (angiography): Depending on the type of vasculitis, diagnosis can also be aided by an X-ray examination (angiography). If the medial and large vessels are affected by vasculitis, physician can represent the vessels with a contrast medium and X-rays, and thus detect possible morphological changes, such as aneurysms.
The treatment of vasculitis varies with each case: the most appropriate type of treatment depends on the extent to which organs are affected and the severity of the disease.
Many vasopressin medications are available to suppress the immune system (immunosuppressants). In the treatment of vasculitis are especially essential:
- Cortisone preparations
- In special cases it may be that the normal dose of the medication is not sufficient to control the possible evolution of the vasculitis. Experimental forms of treatment of vasculitis may also be used, which may be very successful in certain cases of severe vasculitis (such as Behcet’s disease with pulmonary vascular involvement). One of these therapies is high-dose chemotherapy and stem cell transplantation. However, it is advisable to follow these treatments in hospitals with many years of experience in stem cell transplantation.