After Tara Palmer-Tomkinson’s death what is the rare autoimmune disease the socialite was battling, and how can it prove fatal?
The original It Girl was found dead at her West London home on Wednesday, three months after revealing she was battling a brain tumour and rare autoimmune disorder
TARA Palmer-Tomkinson was found dead in her West London flat yesterday, just three months after revealing her battle with ill-health.
The 45-year-old was diagnosed with a benign brain tumour in January last year, but it was another disease ravaging her body, attacking her from the inside out, that posed a greater threat.
The original It Girl confided in pals she was worried the conditions could kill her.
Doctors diagnosed Tara with a pituitary brain tumour - a non-malignant growth just behind the eyes that affects the production of hormones in the body and can result in infertility and a loss of sex drive.
But, further blood tests uncovered another sinister disorder.
While the brain tumour was unlikely to pose a threat to her life, the rare autoimmune condition specialists identified was potentially fatal.
The condition, which involved anti-neutrophil cytoplasmic antibodies (ANCAs), causes the immune system to attack the body rather than protect it.
Left untreated the condition can "eat away" at the lungs, kidneys, and other parts of the body.
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WHAT ARE ANCAs?
The blood is made up of two distinct parts - solids and fluid.
Red and white blood cells make up the solids, while plasma is the fluid.
Plasma cells produce antibodies, that are used by the body's immune system to fight bacteria and viruses.
Antibodies are able to recognise the germs as foreign bodies and neutralise them.
But, anti-neutrophil cytoplasmic antibodies (ANCAs) are a specific group of abnormal antibodies.
Rather than protect the body, they attack cells and tissues.
ANCAs attack white blood cells, which then attack the walls of small vessels in different body tissues and organs.
This can cause vasculitis.
WHAT IS ANCA VASCULITIS?
Vasculitis is inflammation of the blood vessels, caused by white blood cells attacking part of the body tissue.
There are many different causes of the condition, of which autoimmunity is one.
In these cases the immune system turns against the body and causes vasculitis.
ANCA vasculitis is where these abnormal antibodies attack the body's cells and tissues,
What are the symptoms?
In different people ANCA vasculitis will attack different parts of their body.
The condition can attack the kidneys, skin nerves and lungs, the nose, eyes and ears.
Vasculitis in the skin can cause red spots, in the lungs or nose it can cause bleeding.
In the nerves it causes tingling and weakness.
In the eyes vasculitis causes redness and itching, while in the kidneys it causes blood and protein to leak into the blood, and ultimately can cause kidney failure.
Who gets vasculitis?
Vasculitis is very rare, with around one case in every 50,0000 people.
Typically patients are around 55 years old, though in very rare cases it will be diagnosed at a younger age.
How long does the condition last?
While in some people the condition can be short-lived and cured by treatment, in others it is a long-term disease.
Many people can live long and healthy lives, and enjoy long periods where their disease is largely manageable.
Patients will have to undergo regular check-ups, because there is always a risk the disease will return.
How do doctors detect vasculitis?
There are a range of tests doctors can use to identify and diagnose vasculitis.
The type of test you have will likely depend on which part of your body is affected by the symptoms.
A blood test is used to identify ANCA, while further blood and urine tests can see if the condition is affecting the kidneys.
And chest x-rays and scans can check for lung problems.
How is it treated?
Treatment for the disease can be tricky, and will likely involve a number of doctors, depending on how many body parts are affected.
The aim of the treatment is to reduce the inflammation and swelling to protect the body's organs from damage.
The specific treatment will vary depending on the part of the body the disease is attacking.
Various drugs that reduce the immune system's ability to attack itself, as well as certain antibiotics and drugs that remove ANCA from the blood are options.
In severe cases where patients' kidneys are affected by ANCA vasculitis, transplant can be an option to combat kidney failure.
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