Vasculitis

Vasculitis is an inflammation of the blood vessels.  It is commonly associated with diseases such as SLE, scleroderma, ulcerative colitis, rheumatoid arthritis and dermatomyositis. Immune complexes are formed when antigens bind to antibodies.  If not excessive, and assuming a healthy immune response, these complexes are usually cleared from the circulation by the phagocytic system.  These responses are usually delayed, occurring hours or even days after exposure to the antigen, from whatever source.  When these complexes persist in the circulation, their interaction with platelets results in the release of vasoactive amines.  Vasoactive amines cause increased vessel permeability, platelet aggregation (clotting) and initiation of the arachidonic acid cascade (inflammation).  These immune complexes can also settle out within the postcapillary venules, with subsequent tissue inflammation, irritation and damage by complement activation, more inflammatory cells coming to the area and destructive lysosomal enzyme release.

Liver diseases such as chronic hepatitis, alcoholic cirrhosis and biliary cirrhosis are associated with lowered hepatic clearance and increased circulating immune complexes.

Causes and Development

Drugs, complement deficiencies, viral, bacterial and protozoan infections have all been implicated.

Signs and 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 only 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 ("malaise"), 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 ("petechiae"), usually most numerous on the legs.  When the spots are larger, about the size of the end of a finger, they are called "purpura".  Some look like large bruises.  These are the most common vasculitis skin lesions, but hives, an itchy lumpy rash, and painful or tender lumps can occur.  Areas of dead skin can appear as ulcers (especially around the ankles), small black spots at the ends of the fingers or around the fingernails and toes ("nail fold infarcts"), or gangrene of fingers or toes.
• Joints
  Aching in joints and a frank arthritis with pain, swelling and heat in joins.  Deformities resulting from this arthritis are rare.
• Brain
  Vasculitis in the brain can cause many problems, from mild to severe.  They include headaches, behavioral disturbances, confusion, seizures and strokes.
• Peripheral Nerves
  Peripheral nerve symptoms may include numbness and tingling (usually in an arm or a leg, or in areas which would be covered by gloves or socks), loss of sensation or loss of strength (especially in the feet or hands).
• 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.
• Heart
  Vasculitis in the coronary arteries is unusual in lupus.  If it occurs, it can cause a feeling of heaviness in the chest during exertion ("angina"), which is relieved by rest.  Heart attacks rarely occur as a result of vasculitis.
• 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.  Inflammation can occasionally lead to scarring of lung tissue with chronic shortness of breath.
• Kidneys
  Vasculitis is not common in kidneys of people with lupus, even those who have lupus nephritis.  It may not cause any symptoms, although most patients with renal vasculitis have high blood pressure.
• Eyes
  Vasculitis involving the small blood vessels of the retina can occur in cases of lupus.  The retina is a tissue at the back of the eye which contains cells that have to be activated to form a visual image.  Vasculitis of the eyes occasionally causes no symptoms but usually there is visual blurring which comes on suddenly and stays, or a person may even lose a portion of their vision.  In other non-lupus types of vasculitis, such as temporal arteritis, there is sudden loss of part or all of the vision in one eye, usually accompanied by sever headache.

Treatment and Prevention

Treatment revolves around the removal or reduction of circulating immune complexes, dampening down the inflammatory response, stabilising lysosomal cell membranes and improving circulation to damaged tissues.

Sometimes drugs, hidden infections and food allergens increase the level of circulating immune complexes.  Exposure to these should be reduced.  Avoid temperature extremes, as cold can precipitate immune complexes.