Chronic Vasculitis

Vasculitis is a general term for a group of diseases that involve inflammation of blood vessels. The inside of the walls of blood vessels get inflamed.

Blood vessels of all sizes may be affected, from the largest vessel in the body to the smallest blood vessels in the skin. The size of blood vessel affected varies according to the specific type of vasculitis.

Causes

Though the causes of most vasculitis are currently unknown, it is clear, however, that the immune system plays a critical role in the tissue damage caused by vasculitis. The immune system, normally a protective organ of the body, becomes “hyperactive” in vasculitis because of some unknown stimulus, leading to inflammation within the body’s tissues. Inflammation in blood vessel walls leads to narrowing of the vessels. The resulting inadequate blood supply to a particular tissue or organ results in damage. Several things can happen to an inflamed blood vessel. If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue. These areas will appear as small red or purple dots on the skin. If a larger vessel is inflamed, it may swell and produce a nodule, which may be felt if the blood vessel is close to the skin surface.

Vasculitis can occur in many different illnesses. Some of the illnesses that can cause vasculitis are:

Infections

  • Autoimmune Diseases
  • Lupus
  • Henoch-Schonlein Purpura
  • Rheumatoid Arthritis
  • Polymyalgia Rheumatica
  • Scleroderma
  • Wegener’s Granulomatosis
  • Temporal Arteritis
  • Cryoglobulinemia

Erythema Nodosum

Urticarial Vasculitis

Tumors

  • Leukemia
  • Lymphoma

Vasculitis can also occur by itself without any obvious associated infection or other illness.

Symptoms

Vasculitis can cause many different symptoms, depending upon what tissues are involved and the severity of the tissue damage. Some patients are not ill and notice occasional spots on their skin. Others are very ill with systemic symptoms and major organ damage.

A list of symptoms based on the tissues in which vasculitis occurs include:

Systemic symptoms: Fever, generally feeling bad, muscle and joint pain, poor appetite, weight loss, and fatigue. This set of complaints can occur in many illnesses and is not specific to vasculitis.

Skin: Red or purple dots, usually most numerous on the legs. Some look like large bruises. These are the most common vasculitis skin lesions, but hives, itchy lumpy rash, and painful or tender lumps can occur. Areas of dead skin can appear as ulcers or small black spots at the ends of the fingers or around the fingernails and toes.

Joints: Aching in joints and a frank arthritis with pain, swelling and heat in joints.

Peripheral Nerves: Peripheral nerve symptoms may include numbness and tingling in an arm or a leg.

Intestines: Inadequate blood flow in the intestines can cause crampy abdominal pain and bloating. If areas in the wall of the intestine develop gangrene, blood will appear in the stool. If the intestinal wall develops a hole (called a “perforation”), surgery may be required.

Lungs: Vasculitis in this tissue can cause pneumonia-like attacks with chest x-ray changes that look like pneumonia, and symptoms of fever and cough.

Blood abnormalities, which often occur when vasculitis is present, include an elevated sedimentation rate, anemia, a high white blood count and a high platelet count. Blood tests can also be used to identify immune complexes or antibodies that cause vasculitis in the circulation and measure whether complement levels are abnormal. These tests take several days to complete. For abdominal symptoms, one may need to get ultrasound or CT scans of the abdomen, or other special x-rays to see the intestines.

Treatment

The choice of treatment for vasculitis depends on the severity of the vasculitis. Many cases of vasculitis do not require treatment. For example, a few spots on the skin now and then (if not combined with other symptoms) may not require any medications.

For more severe cases, Steroid are required. Cortisone-type medications, such as Prednisone, Prednisolone, or methylprednisolone (Medrol) are given as the initial treatment.

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