What is Vasculitis?

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What is vasculitis?

Vasculitis means inflammation of the blood vessels.
Blood vessels transport oxygen and nutritional substances from the heart to the body tissue and return the blood back to the heart.
In the second circulation the heart pumps the blood to the lungs. In the lung capillaries the blood is saturated with oxygen, after which it returns to the heart.
Blood vessels that carry the blood away from the heart are called arteries. Blood vessels that return the blood to the heart are called veins.
Starting from the heart, blood vessels can be divided in large arteries, medium-sized and small arteries, arterioles, capillaries, small and large veins.
The type of vasculitis not only depends on the type of blood vessel affected, but also on the cause (mostly unknown) and the type of inflammation. In short, there is a great variety in disease patterns. They are hard to classify and often overlap. That is why disorder subdivisions vary greatly.
It is impossible to describe all types of vasculitis, but to be complete, we refer to a classic subdivision of Cupps and Fauci (National Institute of Health, USA).
 

Causes of vasculitis
Although the actual cause of most forms of vasculatic diseases remains unknown, certain blood components are suspected to be responsible. Immune complexes play an important role. These are antibodies connected to antigens that whirl around in the blood and attach themselves to the lining of the small blood vessels where they cause inflammation. Such immune complexes may consist of viruses or parts of viruses together with normal antibodies, but they may also consist of autoantibodies that bind with own antigens. The rheumatoid factor is also commonly found in immune complexes. In some cases, autoantibodies attack the lining of the blood vessels directly.
In various connective tissue diseases, and especially in lupus, vasculitis is caused by the formation of immune complexes and autoantibodies.

What is systemic vasculitis?
Systemic vasculitis is a type of vasculitis that may affect any part of the body. It is always a very serious disorder, sometimes with a less favourable outcome, and it can be life threatening. In most cases, intensive treatment is necessary.
The gravity and disease pattern of the different types of vasculitis vary greatly, with respect to the internal organs in which blood vessels are inflamed. If blood vessels are inflamed, the organ itself will undoubtedly be affected too. As such, a patient with polyarteritis and with inflammation of the kidneys and the brain is not comparable to a patient with skin involvement only.

Different types of vasculitis
Classic polyarteritis nodosa
This is a necrotic (local dying off of tissue) type of vasculitis of the small and medium-sized arteries. Every organ in the body may be affected but there is a predisposition to involve the kidneys and internal organs, next to the muscles and joints. The average age of the onset of the disease is 45, and it occurs 3 times more in males than in females. Enkele vormen van vasculitis

Allergic angiitis and granulomatosis (Churg-Strauss Syndrome)
Differs from polyarteritis nodosa by a more frequent involvement of the lung capillaries, by affecting a wider range of blood vessels and by the formation of granulomas (small and nodular inflammatory lesions) in and round the blood vessels. All of this is accompanied by a serious form of asthma, and an increased number of eosinophiles in the blood (type of white blood cell important in allergies) and infiltration of eosinophiles in the body tissue. The patients affected are of the same age group as in polyarteritis nodosa, with a slightly higher male predominance. The disease is rare but exact figures are not known.

Hypersensitivity vasculitis
This is a group of diseases in which various clinical syndromes occur which have inflammation of the small blood vessels, including the small arteries, capillaries and venules, in common. Especially the latter are likely to become involved (venulitis). Although all organs may be involved, manifestations of the skin will dominate the clinical symptoms. The outcome of the disease tends to be positive, as there is an indication of the cause. This can be a drug, a foreign protein or a bacterium. Other immune complexes may also be of significance.

Wegener's Granulomatosis
The cause of this disease is not known. It is a combination of a necrotic granulomatous vasculitis of the upper and lower respiratory tract, glomerulonefritis (inflammation of the kidneys) and varying degrees of inflammation of the small blood vessels.
It is a rare disease with a slight male predominance. First manifestations are situated at the level of the upper respiratory tract with sinus inflammation, inflammation of the nose, nose blocking, inflammation of the ear, etc. Occasionally there is inflammation of the dental gums, nose bleeds, throat ache. In most cases the lungs are involved although only one third of the patients complains about it. Those suffering from it, complain about coughing, shortness of breath and chest pains.
Very typical for Wegener's granumatolosis is the presence of ANCAs. These are autoantibodies directed against components in the cytoplasm of white blood cells. It is a very serious disorder requiring very intensive treatment. The prognosis of the disease has changed thanks to cyclofosphamide (Endoxan).

Giant Cell Arteritis
Affects the main arteries and can be divided into 2 large groups.
Temporal arteritis is a type of systemic arteritis of the large and medium-sized arteries and occurs mainly in older people. The head arteries are commonly inflamed, occasionally causing sudden blindness. Other arteries throughout the body may exceptionally be affected, leading to insufficient oxygenated blood supply to the limbs and/or internal organs.
In Takayasu arteritis, the large and medium-sized arteries are inflamed and become narrower, often involving the aortic arch and its branches. Patients tend to be young females. The cause is unknown.

Thromboangiitis obliterans or Buerger's Disease
Type of vasculitis that may affect, narrow and eventually obstruct the medium-sized and small arteries and veins, mainly in the hands and feet. There is a clear association with tobacco use. Hence, giving up smoking is the only treatment and is mostly effective.

Behçet's disease
Classic symptoms are episodes of aphthous oral ulcers, genital ulcers as well as recurrent eye inflammation (uveitis). The underlying cause is a vasculitis that affects mainly the small veins. Behçet's disease, however, may occur in all types of organs because of its ability to affect blood vessels of nearly all sizes and types.

Erythema nodosum
Characterised by returning groups of painful red nodular (knob-like) subcutaneous lesions at the level of the lower legs, sometimes also located elsewhere. In 50 % of the patients no cause is found and lesions disappear spontaneously. In others, an underlying cause is found. The cause may be tuberculosis, a streptococcus infection or other infections, infections of the intestines, sarcoidosis, Behçet's disease.

Treatment
Systemic vasculitis requires in most cases intensive treatment with corticosteroids, sometimes combined with immunosuppressive drugs like azathioprine (Imuran) or cyclofosfamide (Endoxan). Treatment has to be individualised according to the extent, seriousness and evolution of the involved organs
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