The full name of your uncle's condition is "polyarteritis nodosa." I
have gathered some information about the disease, and I'll try to help
give your uncle a glimpse of what his prospects are likely to be. I'm
in a good position to understand your uncle's concerns: I was
diagnosed with polyarteritis nodosa in 1998.
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-= WHAT IS POLYARTERITIS NODOSA? =-
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Polyarteritis nodosa is described as "a systemic necrotizing
vasculitis of small and medium-sized arteries." The disease is also
known by several other names: periarteritis nodosa, PAN, Kussmaul's
disease, microscopic polyangiitis, and microscopic polyarteritis.
What does "systemic necrotizing vasculitis" mean? When a disease is
said to be "systemic," this means that the disease process is not
localized to just one area of the body, but is widespread.
"Necrotizing" refers to the destruction of bodily tissue, rendering it
essentially "dead." "Vasculitis" is the inflammation of a blood vessel
(or a lymphatic vessel). So, as we can see by looking at the
components of the term, systemic necrotizing vasculitis is a full-body
condition involving the inflammation of blood vessels, which can lead
to serious damage to the tissue of the vessel walls.
Stedman's Medical Dictionary gives this definition:
"polyarteritis nodosa
segmental inflammation, with infiltration by eosinophils, and necrosis
of medium-sized or small arteries, more common in males, with varied
symptoms related to involvement of arteries in the kidneys, muscles,
gastrointestinal tract, and heart"
("Eosinophils" are a specific type of white blood cell. "Necrosis"
means the death of living tissue.)
Here are some good descriptions of the disease in simple terms:
"Polyarteritis nodosa is a disease of unknown cause that affects
arteries, the blood vessels which carry oxygenated blood to organs and
tissues. It occurs when certain immune cells attack the affected
arteries.
The condition affects adults more frequently than children. It damages
the tissues supplied by the affected arteries because they don't
receive enough oxygen and nourishment without a proper blood supply.
In this disease, symptoms result from damage to affected organs, often
the skin, heart, kidneys, and nervous system."
AllRefer Health: Polyarteritis Nodosa
http://health.allrefer.com/health/polyarteritis-nodosa-info.html
"Polyarteritis nodosa (PAN) is a specific form of the autoimmune
disease known as vasculitis, which involves inflammation of the blood
vessels. In polyarteritis nodosa inflammation occurs in the medium
sized arteries, impairing blood flow through the vessels and to the
surrounding tissues. The disease usually develops at 40 to 50 years of
age. Men are three times more likely than women to develop it...
The cause of polyarteritis nodosa is unknown. It is clear, however,
that the immune system plays a critical role in the tissue damage
caused by the disease. In polyarteritis nodosa the immune system
produces antibodies that attack the body, causing inflammation to
develop in the blood vessels. Inflammation in blood vessel and artery
walls leads to narrowing of the vessels. The blood vessels become
leaky or clogged, disrupting blood flow to nerves, organs and other
parts of the body. Inadequate blood supply to a particular tissue or
organ can result in permanent damage such as loss of pulse points,
blindness and pulmonary failure. Any organ or combination of organs in
the body can be affected."
Caring Medical
http://www.caringmedical.com/symptoms/condition.asp?condition_id=973
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-= HOW IS POLYARTERITIS NODOSA TREATED? =-
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Your uncle may have been told that polyarteritis nodosa is incurable.
That is the bad news. However, the good news is that, although we
don't know how to cure the disease, effective treatments are
available, and patients who receive treatment can live for many years.
The two families of drugs are most commonly used in combating
polyarteritis nodosa are corticosteroids and alkylating/antineoplastic
agents.
Corticosteroids (or glucocorticoids) are sometimes called "cortisone
drugs." These are drugs that fight inflammation and alter the body's
immune response. They are not the same thing as anabolic steroids, the
drugs sometimes used by bodybuilders and athletes. The corticosteroids
most commonly used to treat PAN are prednisone, prednisolone, and
methylprednisolone.
Alkylating antineoplastic drugs inhibit the growth and proliferation
(multiplication) of cells. "Alkylating" refers to the replacement of
hydrogen with an alkyl group within a molecule, targeting the DNA in
cells (an alkyl group is a specific kind of hydrocarbon chain).
"Antineoplastic" describes a drug that is intended to slow down or
prevent the abnormal growth of new cells. Drugs of this type are often
used in cancer chemotherapy, but polyarteritis in NOT a cancer. Among
the alkylating antineoplastic drugs that are used in treating PAN are
cyclophosphamide and methotrexate.
You have mentioned that your uncle has cirrhosis. It is possible that
his PAN could be related to the hepatitis virus. If this is the case,
there is a third type of drug treatment that may be enlisted:
"Until recently, treatment for all cases of polyarteritis nodosa
started with high doses of corticosteroids, which increased the
survival rate appreciably.
For those patients whose disease is caused by hepatitis B or C,
treatment now also includes injections of interferon alpha, an
antiviral drug that has been available for a number of years.
The newer antiviral drug that is being used is lamivudine, marketed as
Epivir which is effective in treating hepatitis B. While interferon
must be given by injection, the new drug can be taken orally, which
makes its use much easier.
Not all patients with polyarteritis nodosa can be helped by these
drugs. They are effective only when the hepatitis viruses cause the
condition."
Tri-City Herald: New Drug Improves Outlook for Polyarteritis Nodosa
http://archive.tri-cityherald.com/HEALTH/medicine/med26.html
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-= IF I HAVE POLYARTERITIS NODOSA, WHAT ARE MY CHANCES OF DYING? =-
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It is essential that this disease be treated aggressively. Without
treatment, polyarteritis nodosa can be fatal within a few months of
onset. However, treatment with corticosteroids and/or antineoplastic
drugs dramatically improves the outlook.
"Prognosis:
PAN carries a high mortality rate when untreated. Nearly one half of
patients die within the first 3 months of onset. Corticosteroid
treatment improves the 5-year survival rate to 50-60%. When it is
combined with other immunosuppressants, the 5-year survival rate may
increase to greater than 80%."
eMedicine: Polyarteritis Nodosa
http://www.emedicine.com/NEURO/topic314.htm
"Before the use of glucocorticoids, five-year survival of individuals
with polyarteritis nodosa was ten percent if untreated. Today the
five-year survival rate approximates 96 percent with the use of
glucocorticoids and other immunosuppressive agents, such as
cyclophosphamide. Patients with systemic involvement usually require
high dose glucocorticoid therapy, frequently for years. Patients with
primarily cutaneous involvement will often achieve remission within
three to six months on lower doses of glucocorticoids."
Dermatology Online Journal: Polyarteritis Nodosa
http://dermatology.cdlib.org/DOJvol7num1/NYUcases/polyarteritis/kim.html
"The course of illness is variable, with some patients responding
promptly to therapy and tapering off of treatment over many months,
whereas others require long-term treatment...
With proper treatment, at least 70 percent of patients survive for 10
years or more after diagnosis, often recovering completely. After
successful treatment, the risk of relapse is only about 10 percent,
although damaged blood vessels may cause problems (for example, a
heart attack) long after the inflammation has resolved. Without
appropriate treatment, only 10 percent of patients survive for five
years after diagnosis."
InteliHealth: Polyarteritis Nodosa
http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/24696.html
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-= MORE INFORMATION ON POLYARTERITIS NODOSA -=
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Here are some articles that will provide an in-depth understanding of
PAN, in layman's terms:
Johns Hopkins Vasculitis Center: Polyarteritis Nodosa
http://vasculitis.med.jhu.edu/typesof/polyarteritis.html
eMedicine: Polyarteritis Nodosa
http://www.emedicine.com/NEURO/topic314.htm
Medline Medical Encyclopedia: Polyarteritis Nodosa
http://www.nlm.nih.gov/medlineplus/ency/article/001438.htm
Savvy: Polyarteritis Nodosa
http://www.blackandwhite.org/polyart/index.shtml
Quite a bit of useful information is available on the website of the
Polyarteritis Nodosa Research and Support Network:
The Polyarteritis Nodosa Research and Support Network: Articles
http://www.angelfire.com/pa3/autoimmunedisease/Articles.htm
The Polyarteritis Nodosa Research and Support Network: Hospitals
http://www.angelfire.com/pa3/autoimmunedisease/Hospitals.htm
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Google search strategy:
Google Web Search: "polyarteritis nodosa"
://www.google.com/search?hl=en&ie=UTF-8&oe=UTF-8&q=%22polyarteritis+nodosa
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Please keep in mind that Google Answers is not a source of
authoritative medical advice, nor a substitute for the services of a
physician. The material I've presented above should not be viewed as a
diagnosis, but is intended for informational purposes.
I hope this helps! If anything is unclear, please request
clarification; I'll be glad to offer further assistance before you
rate my answer.
Best wishes,
pinkfreud |
