for those with Sjogren's syndrome

[Guest guest]

HELEN BRANSWELL

Canadian Press

Monday, October 07, 2002

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TORONTO (CP) - A surprise finding by researchers investigating a

suspected link between Type 1 diabetes and multiple sclerosis may

help people with a highly irritating condition known as Sjogren's

syndrome.

The team of scientists from the Hospital for Sick Children has

discovered a protein that is critical for the development of

Sjogren's, an autoimmune disease that attacks the body's moisture-

producing glands. " We were looking for gold and we struck oil, "

explained senior investigator Dr. Dosch of the work, which is

published in the current issue of the medical journal The Lancet.

Dosch and his team are hopeful that the process which triggers

Sjogren's can be turned off - and the damage done by the disease

essentially reversed - by vaccinating people who suffer from it. They

even have a prototype vaccine they hope can be put into human trials

in a year or so.

The process works in mice, but more research is needed on the human

form before clinical trials can be undertaken.

" It even worked late in the disease and stopped it and even made it

go almost normal, " Dosch said in an interview. " It comes out of left

field. I mean, nobody would have ever guessed. "

The team is working with Dr. Arthur Bookman, a rheumatologist who

runs a Sjogren's clinic at Toronto Western Hospital, to recruit

people suffering from the disease. (Bookman is also a co-author of

the study.)

They need to do further study of the role of the protein, ICA69, in

the human disease to determine whether the reaction to it is the same

across the board. They plan to do that by testing blood samples from

between 70 to 100 Sjogren's patients.

If it is, the team will seek approval for trials testing the vaccine

on humans.

Theresa Spence, who has suffered from Sjogren's syndrome for more

than 25 years, is thrilled at the news.

" Isn't that exciting? And that it could be reversed? It's amazing, "

says Spence, a Toronto woman in her 50s.

The disease's trigger is not known, though some suspect it is a

virus. Whatever the cause, it prompts the body's immune system to go

haywire. Instead of attacking foreign invaders like bacteria and

viruses, it attacks ICA69, which is found in moisture-producing

glands like tear ducts and salivary and sweat glands.

The result: the glands stop working. Excruciatingly dry eyes and dry

mouth are only the beginning of the problems.

Spence must use artificial tears to keep her eyes moist. She can't

swallow most foods without the help of a drink. (Bookman said a good

way to diagnose Sjogren's is to ask a suspected sufferer to chew and

swallow a soda cracker. " They never can. " )

Spence doesn't sweat, so she has to avoid activities in which she'll

become overheated. " Once I've heated up, I stay like a solid little

brick for ages. It's just awful. "

The problems might seem minor. They are anything but.

" I've had tonnes and tonnes of cavities. Extensive dental work to try

and save teeth, " Spence explained.

" They considered implants because I've lost teeth at the back, but

because I've been on steroids so often I have bone loss so (that's

not an option). And false teeth are not a good thing for me, because

I would just have irritation " because of the lack of moisture.

Sjogren's often progresses to attack internal organs and the body's

blood vessels. Spence has suffered a lung embolism, has had to have

her spleen removed, and has endured several bouts of bacterial

pneumonia which were exacerbated by the fact she has no spleen.

Spence and all Sjogren's patients walk around knowing one in 10 of

them will go on to develop something far more serious.

" The biggest worry of mortality in these patients is lymphoma, "

Bookman said. " The immune system gets so revved up it takes off on

its own and becomes malignant. "

It's estimated that one in 100 people - the vast majority of them

women - suffer from Sjogren's syndrome. Many of them develop it as a

side-effect of other autoimmune diseases, such as lupus and

rheumatoid arthritis, Bookman said.

Despite that, the disease has no public profile and many doctors are

not familiar with it.

" The average patient has to see three doctors and it takes up to

three years before they can find a diagnosis, " Bookman said.

" It's an orphan disease in a way, " Dosch agreed.

" It doesn't kill. It's a real nuisance. Some people even go blind.

But there's no understanding of the disease. It's hard to diagnose

and there's no therapy. "

This accidental discovery that ICA69 is the target of the autoimmune

attack may lead to one. The idea would be to desensitize the immune

system to the protein, essentially teaching the body that ICA69 poses

no threat.

Dosch is hopeful that as part of the bargain, science will learn a

lot more about shutting down other debilitating autoimmune processes.

Researchers have been working on developing vaccines for some of

these diseases, but have never managed to translate success in animal

trials into success in humans.

Sjogren's should be an easier task, he said, because it appears that

only one protein is targeted by the autoimmune response. In diseases

like lupus and rheumatoid arthritis, multiple proteins are involved.

" This may be the door to allow us to learn how to translate animal

data into humans, " Dosch said. " And it may be as simple as finding

the right dose. "