Tuesday, July 13, 2010

No Sailing in these Vessels...


Vasculitis is inflammation which occurs in the blood vessels. Inflammation occurs as tissue is damaged by mostly white blood cells entering the tissues. White blood cells circulate and serve as the main defense against infections. Normally, white blood cells destroy bacteria, fungi and viruses. However, in people with autoimmune conditions, they damage normal tissue when they invade it.

Vasculitis can affect:  very small blood vessels (capillaries), medium-size blood vessels (arterioles or venules), or large blood vessels (arteries or veins)

How Does Vasculitis Damage The Body?

Several things can happen to an inflamed blood vessel.

v  For a small vessel, it may break and produce tiny areas of bleeding in the tissue…which can appear as small red or purple dots on the skin.
v  For a larger vessel, it may swell and produce a lump or nodule…which may be felt if the blood vessel is close to the surface of the skin.
v  If there is narrowing of the blood vessel, then blood flow is reduced.  Occasionally, there may be a total blockage caused by a blood clot which forms at the site of inflammation.
v  If blood flow is reduced or stopped, the tissues affected can die.  This can progress to gangrene if not treated.


What Causes Vasculitis?

Vasculitis can be caused by many things, such as:

Ø  Infection of the blood vessel walls which is rare. When it occurs, bacteria, viruses or fungi infect the blood vessel and the white blood cells move in to destroy the infectious agents and damage the blood vessel in the process.  This is a serious condition and requires prompt medical treatment.

Ø  Immune or "allergic" reaction within the vessel walls.  This cause of vasculitis is more common.  The substances which trigger allergic reactions are called antigens. These antigens cause the body to produce proteins called antibodies which are supposed to bind to the antigen get rid of it.

Ø Antigens and antibodies which bind together are called immune complexes. Two primary ways in which immune complexes destroy antigens are:   by attracting white blood cells to digest the antigen, or  by activating other body substances to help destroy the antigens.

Ø  Unfortunately, some immune complexes do not serve their purpose of destroying antigens. Instead, they remain too long in the body and circulate in the blood and deposit in tissues. They commonly accumulate in blood vessel walls, where they cause inflammation.  It is likely that some white blood cells (cytotoxic cells) which kill infectious agents can also accidentally damage blood vessels and cause vasculitis.

Ø Vasculitis Caused By Lupus.  In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens. A recently discovered antibody, ANCA (anti-neutrophil cytoplasm antibody), can cause vasculitis in some individuals.

Signs and symptoms by type of vasculitis

General signs and symptoms common to most vasculitis types vary depending on which blood vessels and, as a result, which organ systems are affected.  However, general signs and symptoms that most people with vasculitis experience include: fever, fatigue, weight loss, muscle and joint pain, loss of appetite, and nerve problems, such as numbness or weakness.

Ø Behcet's syndrome. This condition causes inflammation of your arteries and veins, and often appears in your 20s and 30s. Signs and symptoms include mouth and genital ulcers, eye inflammation and acne-like lesions on your skin.
Ø Buerger's disease. Also called thromboangiitis obliterans, this condition causes inflammation and clots in the blood vessels in your extremities. Signs and symptoms can include pain in your hands, arms, feet and legs, and ulcers on your fingers and toes. This disorder is strongly associated with cigarette smoking.
Ø Central Nervous System Vasculitis. There are two types -- Primary angiitis of the central nervous system (PACNS) and Benign angiopathy of the central nervous system (BACNS). 
·        The main symptoms include many neurologic symptoms and signs: headache, focal weakness (such as occurs with a stroke), seizures, bleeding within the CNS, confusion, disorders of memory, and altered consciousness. All of these symptoms and signs are non-specific, and can be mimicked by a variety of other conditions. Thus, despite the “benign” part of the designation “BACNS”, some patients with so–called “benign“angiopathy of the CNS sustain significant neurological damage.
·        There are two main differences in the clinical presentations of PACNS and BACNS. First, whereas PACNS patients are more likely to develop symptoms subacutely and remain undiagnosed for months, those with BACNS are more likely to have relatively acute presentations and be diagnosed within weeks of onset (in either case, however, making the diagnosis is challenging). Second, without treatment, patients with PACNS tend to have progressively downhill courses that often lead to death. In contrast, BACNS patients may require less aggressive treatment than PACNS. A common difficulty comes in sorting out which patients are more likely to have clinical courses characteristic of BACNS, and which require the more aggressive treatment approach called for by PACNS.

This is the type of vasculitis that I typically get.  It is very difficult for the doctor’s to realize this is going on as mine is the PACNS form which develops slowly and is more insidious and subtle in its presentation.

Ø Churg-Strauss syndrome. This condition, also known as allergic granulomatosis and allergic angiitis, most commonly affects the blood vessels in your lungs. It's often associated with asthma.
Ø Cryoglobulinemia. This condition is often associated with hepatitis C infections. Signs and symptoms include a rash called purpura on your lower extremities, arthritis, weakness and nerve damage (neuropathy).
Ø Giant cell arteritis. This condition, which occurs in people older than 50, is an inflammation of the arteries in your head, especially your temples. Giant cell arteritis can cause headaches, scalp tenderness, jaw pain while chewing, blurred or double vision, and even blindness. Giant cell arteritis is often associated with polymyalgia rheumatica.
Ø Henoch-Schonlein purpura. This condition is caused by inflammation of the blood vessels of your skin, joints, bowel and kidneys. Signs and symptoms can include abdominal pain, blood in the urine, joint pain, and a rash called purpura on your buttocks, legs and feet. Henoch-Schonlein most often occurs in children, but it can occur at any age.

This is the other form of vasculitis I have experienced.  It usually shows up on my legs and in my kidneys.  It has been in what triggers the damage in the filtering system within my kidneys.  My BUN and creatinine levels are still too high for my rheumatologists liking.

Ø Hypersensitivity vasculitis. The primary sign of hypersensitivity vasculitis is red spots on your skin. It can be triggered by an allergy, most often to a medication or an infection.
Ø Kawasaki disease. Also known as mucocutaneous lymph node syndrome, this condition most often affects children younger than 5 years of age. Signs and symptoms include fever, skin rash and eye inflammation.
Ø Microscopic polyangiitis. This form of vasculitis affects small-sized blood vessels in your kidneys, lungs and skin. Signs and symptoms include skin lesions, fever, unintentional weight loss, glomerulonephritis — inflammation of the small blood vessels in the kidneys — and nerve damage.
Ø Polyarteritis nodosa. This form of vasculitis affects medium-sized blood vessels in many different parts of the body, including your skin, heart, kidneys, peripheral nerves, muscles and intestines. Signs and symptoms include a rash called purpura, skin ulcers, muscle and joint pain, abdominal pain, and kidney problems.
Ø Polymyalgia rheumatica. This condition primarily affects older adults and results in pain and inflammation of the large joints, such as your shoulders, hips and knees. Signs and symptoms include pain and stiffness in the muscles of your hips, thighs, shoulders, upper arms and neck. Polymyalgia rheumatica often occurs in association with giant cell arteritis.
Ø Rheumatoid vasculitis. This type of vasculitis can complicate the course of rheumatoid arthritis and usually occurs in people with a history of severe rheumatoid arthritis. Many different parts of the body, including the eyes, skin, hands and feet may be involved.
Ø Takayasu's arteritis. This form of vasculitis includes the largest arteries in the body, including the aorta, and typically occurs in young women. Signs and symptoms include arm weakness or pain with use (claudication), decreased or absent pulses, lightheadedness, headaches, and visual disturbances.
Ø Wegener's granulomatosis. This condition causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Signs and symptoms can include shortness of breath, nasal stuffiness, chronic sinusitis, nosebleeds and frequent ear infections.

Prognosis for those of us with vasculitis…

The outcomes for people suffering from vasculitis vary with each person.  For many patients, especially if it is confined to the skin, vasculitis may be annoying but will never life-threatening. For these individuals, life can be normal -- or pretty close to it.  On the other hand, for a small number people which suffer from severe vasculitis involving major organ systems…in these cases, damage can occur so rapidly that treatment does not have time to work or the condition may be resistant to treatment. An attack of vasculitis can become quickly fatal or be permanently disabling for those affected.  For the vast majority of us with vasculitis, treatment is very effective. However, the vasculitis may disappear only to reoccur later and require treatment again or it may be suppressed but never really go away, so that some ongoing treatment is always required.

Sorry for being a long post…but it is a lot of information.  For now…my vasculitis is healing.  My headaches are gone and my vision is not being affected too much.  I have a large visual field defect in my right eye’s peripheral field that is permanent but I can see clearly.  I just sometimes walk into walls, doors, tables…cats…broken bricks.  As for the kidney involvement…we don’t know.  Since my labs are still too high, I am being optimistic in that it is taking longer for them to show improvement.  I feel better than I have in a long time and I am hoping to use the “R” word soon.  

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