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Vasculitis

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Vasculitis, or angiitis, belongs to a group of diseases which cause inflammation of the body’s blood vessels. It is a disorder in which the body’s immune system inadvertently targets blood vessels which result in inflammation. All blood vessels are affected by this disease, from the aorta, the body’s largest and main blood vessel, to the veins and arteries, to the smallest blood vessels that connect them - the capillaries.

VASCULITIS CAUSES

Inflammation of a blood vessel causes it to narrow and thicken, making it harder for the blood to circulate through it. In some instances, a blood vessel may become clogged completely (Occlusion), blocking all blood flow to or from it. Because of this blockage, the blood vessel may stretche to the point where its walls weaken and swell (Aneurysm). This condition may eventually cause it to burst (Aneurysm rupture).

Vasculitis, or vasculitides in its plural form, may last for only a short time (acute), but it may also be a long-term affliction (chronic). It can cause blood vessel scarring, and can be extremely severe as to affect and damage nearby organ systems. This disease can lead to serious complications and disability. It may even result in death.

VASCULITIS SYMPTOMS

Vasculitis is considered a rare kind of disease but it can afflict any person, with some age groups more prone to acquiring it. Generally, symptoms of vasculitis include fever, swelling, fatigue, weight loss, lack of appetite, muscle and joint pain, nerve numbness, and a general feeling of ill health, all of which characterize an inflammatory condition. There are various types of vasculitis which affect different sizes of blood vessels and cause a wide range of symptoms.

Large vessel vasculitis such as the Takayasu arteritis targets the aorta and the primary blood vessels branching out from it. People over 40 years old are mostly affected by this type of vasculitis, and are likely to experience the symptoms of leg cramps (Claudication of extremities).

Another large vessel form of the disease is Giant cell arteritis, a chronic form of vasculitis which affects the body’s large and medium blood vessels. Older adults of 50 years and above have a higher risk of acquiring this type of vasculitis, which is characterized by headaches and tenderness in the affected artery region.

Under medium blood vessel vasculitis, Polyarteritis nodosa causes weight loss, purplish skin discolorations (Livedo reticularis), weakness or chronic fatigue, and painful testicles. Wegener’s granulomatosis, on the other hand, causes a flattening of the nasal septum (saddle nose deformity), and inflammation of the nasal and mouth passages.

Other medium vessel vasculitides include Kawasaki disease, which afflicts children with fever, fissured lips, an red and swollen pharynx, strawberry-like tongue, and inflammation and swelling of the palms and soles of the feet, as well as rashes and inflammation of the cervical lymph nodes (Cervical lymphadenopathy); Isolated CNS vasculitis, meanwhile, affects the central nervous system.
Vasculitis
Image: Vasculitis

VASCULITIS COMPLICATIONS

Vasculitis also affects the small blood vessels, such as in Churg-Strauss arteritis which inflames the vessels of the lungs and skin. It causes asthma and paranasal sinus abnormalities. Microscopic polyarteritis causes inflammation of the capillaries, arterioles and venules, while Hypersensitivity or allergic vasculitis targets children from the age of 16 onwards. This type of vasculitis is believed to be a drug-related allergy, and it causes a painful skin rash (Palpable purpura), or a red bumpy rash (Maculopaular rash) on the afflicted person.

The Henoch-Schonlein pupura exhibits the formation of lesions in small blood vessels and is characterized by Palpable purpura of the legs and buttocks, gastrointestinal bleeding, the presence of blood in the urine, and abdominal pain (Bowel angina). It usually afflicts people below the age of 20. Essential cryoglobulinemic vasculitis is caused by a hepatitis C infection and results in protein precipitation in small blood vessels.

Some vasculitides can be secondary to other disorders, such as systemic lupus (a chronic autoimmune disease), rheumatoid arthritis, or Behcet’s disease (a chronic blood vessel inflammation). Sometimes the onset of vasculitis may follow a viral infection, such as hepatitis B and C, or HIV.

There are some types of vasculitides whose causes remain unknown. They are referred to as primary vasculitides. Medical research is still searching for the singular cause of this disease. There are strong theories pointing to the presence of vast amounts of antigens in the circulatory system. Antigens are foreign or protein molecules which mimic an immune response. They cause the immune system to produce antibodies which then try to eradicate the antigen, unfortunately affecting the blood vessels in which the antigens travel, as well.

Vasculitis is also believed to be secondary to viral infections, like hepatitis, or bacterial infections like those of the upper respiratory tract. These kinds of vasculitides are referred to as secondary vasculitides. Other theories suggest that the disease may also occur in segments of the population who have a genetic predisposition to acquire vasculitis after having suffered an infection.

VASCULITIS TREATMENT

While most vasculitides are minor illnesses which disappear even without treatment, some will need medication for the patient to get better. Other forms of vasculitis are serious and inflict damage on the body’s main organ systems. These types of the disease may resist treatment and can result in organ failure and even death.

Even with vasculitides that have been successfully treated, there will still be instances of recurrence that may need repetitive therapy. Others are chronic and long-term, and will require regular ongoing treatment.

A tissue biopsy derived from the affected area is the best way of detecting vasculitis. The doctor will determine whether there has been a previous viral or bacterial infection, such as hepatitis or systemic lupus erythematosus. Laboratory tests will involve a complete blood count, creatinine test, muscle enzyme test, urinalysis, liver function test, electrocardiogram, and hepatitis seroloties. Imaging tests like a CT scan, an ultrasound, or an MRI are some non-invasive techniques used to look at large arteries such as the aorta. An Electromyography can test for systemic vasculitis, while an Arteriography can detect large blood vessel vasculitis.

Treatment will depend on the type of vasculitis a patient has, how serious the condition is, and the current state of the patient’s health. Usually the underlying cause of vasculitis is subject to treatment, and the doctor may prescribe steroids and immunosuppressants.

Specific medications also include corticosteroids like prednisone or methylprednisolone (Medrol), cytotoxic drugs to fight inflammation such as azathioprine (Imuran) or cyclophosphamide (Cytoxan), and nonsteroidal anti-inflammatory drugs (NSAIDs) to treat the milder types of vasculitis.


There are no known methods of preventing the occurrence of vasculitis, however, early detection and prompt treatment can reduce symptoms, and prevent or delay the complications that accompany the disease.

VASCULITIS PROGNOSIS

The prognosis for vasculitis will depend on the type and seriousness that has afflicted a patient. The outlook is positive with early diagnosis and treatment of the milder forms of the disease. Sometimes the disease may disappear or the patient may go into remission, at other times it can be a chronic ailment requiring long-term treatment. Severe cases, on the other hand, may not respond to treatment at all, and may result in serious debilitation or even death.

The Vasculitis Foundation is currently holding a clinical study to take a closer look at the different types of vasculitis such as Wegener’s granulomatosis, Takayasu’s artertis, polyarteritis nodosa, microscopic polyangiitis and the Churg-Strauss syndrome. The clinical study will be focusing on the role of genetics and identifying the particular genes that may play a part in each specific type of vasculitis.
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