A 'sneaky' disease that affects mostly older women

Sjogren’s syndrome affects at least 80,000 Israelis, but is under-diagnosed and gets too little attention in medical schools and from drug companies.

By
January 2, 2016 22:28
Prof. Eldad Ben-Chetrit (R) and Prof. Doron Aframian

Prof. Eldad Ben-Chetrit (R) and Prof. Doron Aframian. (photo credit: Courtesy)

What is more natural than the glands in the neck and mouth salivating and those in the hollow of the eye producing tears for lubrication of the cornea? One doesn’t even think of it.

But don’t take these vital processes for granted.

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Between 0.5 percent and 3% of the world population suffers from Sjogren’s (pronounced Shogrin’s) syndrome, which causes dry mouth and/or dry eye, can be accompanied by systemic rheumatic diseases, and can even significantly increase the risk for a cancer of the lymphatic system.

The situation is complicated by the fact that symptoms can be caused by other disorders and even appear as side effects from taking medications or undergoing radiation for head and neck cancers.

Since a variety of medical specialists, from dentists and ophthalmologists to rheumatologists and oncologists, could be involved, and not all are alert to the complicated syndrome, the patient may be diagnosed as long as a decade after she (in nine out of 10 cases) or he complains.

There is no cure, but about 80% have a primary, bearable condition that can be eased with medications and do not involve bodily systems and secondary diseases. The earlier the diagnosis, the better.

One of the highest prevalences of Sjogren’s in the world – almost 3% of the population – can be found in Sweden, possibly for genetic or environmental reasons, but it is likely because of increased awareness of the syndrome. While the 19th-century Polish-Austrian surgeon Johann von Mikulicz-Radecki is usually credited with the first description of the syndrome, it had to wait for Swedish ophthalmologist Dr. Henrik Samuel Conrad Sjogren to give details in his doctoral thesis on the condition that aroused international interest.

Sjogren’s is known today as a multi-system, auto-inflammatory disorder of the exocrine glands (that produce and secrete substances onto an epithelial surface). But little about it is taught in most medical and dental schools, so general medical practitioners often are unable to identify the problem. When a graduate advances into a specialty, the medical resident is often not told about Sjogren’s symptoms relating to another specialty.

SJOGREN’S SYNDROME is a widely under-diagnosed disease,” said Prof. Doron Aframian, head of the department of oral medicine and the salivary gland clinic at the Hebrew University- Hadassah School of Dental Medicine in Jerusalem’s Ein Kerem neighborhood, in an interview with The Jerusalem Post.

“A delay in its diagnosis may have a significant physical, psychological and economic impact on the affected person.”

Aframian was chairman of a recent conference held in Tel Aviv and attended by some 150 experts in several fields to discuss the multidisciplinary nature of Sjogren’s. It was the first conference of its kind held in Israel.

“It appears to involve a number of factors – immunological, genetic, hormonal and possibly infectious from viruses. Its successful management requires a multidisciplinary approach, and the dentist plays an essential role in the diagnosis and treatment of the disease,” he said.

“Those patients who suffer impairment of salivary function have a higher risk of developing oral diseases.

Effective management of their oral health includes giving drugs to boost the production of saliva and preventing and treating of cavities, oral candidiasis (fungus infections) and allergic inflammation in or around the mouth,” explained Aframian, who also directs the dental school’s oral medicine sedation and musculofacial radiology unit and coordinates Sjogren’s treatment at Hadassah University Medical Center.

He has seen patients as young as eight years old, both male and female, but most of them are women over the age of 40 or 50.

Aframian hopes that in the future, the new Sjogren’s center at Hadassah (the only one of its kind in the country) will become a “one-stop shop” where all specialists will see patients in one place and consult with each other.

“It’s a matter of budget. The health funds prefer to send patients to their own experts in a specific field rather than an external center with all relevant specialists together.”

He estimates the number of patients in this country at 80,000, with a prevalence of 0.5% to 1%, but there may be many more due to under-diagnosis. In general, women are more likely – partly due to hormones – to suffer from autoimmune diseases in which the body’s immune system attacks various organs or tissues, he suggested.

“It’s complicated. The disease has no steady pattern. There are some 500 different medications that can affect salivation; as many as 15% of the population suffer from dry-eye symptoms. There is no specific clinical marker or biomarker for the disease. So many patients can fall between the cracks,” he said.

A woman may be treated by her ophthalmologist just for dry eyes, but then suddenly, she also develops dry mouth – or she may lack both, but still have Sjogren’s, Aframian said.

“Not all ophthalmologists know to ask dry-eye patients about the condition of their mouth or dentists know to ask dry-mouth patients if they have dry-eye symptoms.”

As part of the effort to increase awareness of the disorder, Aframian and colleagues have opened a section in the Camoni medical information website (camoni.co.il). There is also an intention to re-establish a Sjogren’s patients’ association after one closed several years ago due to the death of the chairman.

There are several Sjogren drugs on the market and in the basket of health services, but for the most expensive, one has to have been diagnosed positively with a biopsy of minor salivary glands in the lip.

“As a very sneaky disease,” said Aframian. “The biopsy doesn’t always show Sjogren’s, so you can’t get all the drugs available in the basket that you may need.”

PROF. AVI Solomon, a cornea expert at Hadassah University Medical Center, told the Post that of the 600 ophthalmologists in Israel, there are only 30 experts in diseases affecting the cornea covering the front of the eyeball, and three of them are at Hadassah.

The dryness can be alleviated with over-the-counter artificial tears – preferably without artificial preservatives, as these can irritate some eyes.

There are also prescription drugs such as steroids. A very expensive drug called Restasis (cyclosporine, an anti-rejection drug for organ-transplant patients) is beneficial for dry eye, but only those patients who have an official diagnosis of Sjogren’s can get them at a highly subsidized price. Drops reduce friction caused by the lack of tears and this can prevent or reduce redness and actual damage to the cornea.

Many patients suspected of having Sjogren’s undergo the Schirmer’s test, in which a little piece of paper is inserted between the lower eyelid to measure the amount of tears produced by the lachrymal glands. A negative (more than 10 mm. of moisture on the filter paper in five minutes) test result is normal. Both eyes normally secrete the same amount of tears.

To relieve dry eyes, patients may consider undergoing punctal occlusion, a minor surgical procedure to seal the tear ducts that drain tears from the eyes; silicone or collagen plugs are inserted into the ducts for a temporary closure. Those made from collagen eventually dissolve, but silicone plugs stay in place until they fall out or are removed. Some ophthalmologists use a laser to permanently seal the tear ducts.

Stanford University scientists abroad have even developed a device that electronically stimulates tear production to benefit dry-eye patients. The device, 16 mm. long, 3 or 4 mm. wide and 1 to 2 mm. thick, was implanted beneath the inferior lacrimal gland in rabbit eyes.

Activated wirelessly, it was shown to increase the production of tears by nearly 57%, according to a study just published in the Journal of Neural Engineering. The next phase of the research will be to evaluate the “quality” of the tears produced, as in addition to volume, protein and lipid content are important. The device is currently undergoing clinical trials for approval by the US Food and Drug Administration, and it could reach the US market in 2016.

INTERNAL MEDICINE specialist Prof. Eldad Ben-Chetrit, head of the rheumatology unit at Hadassah University Medical Center, who spends much time diagnosing and treating Sjogren’s patients suffering from systemic conditions, not only dry eyes or mouth, said a number of autoimmune diseases can develop in the syndrome. The most prominent are rheumatoid arthritis (RA) and lupus erythematosus. RA is a chronic inflammatory disorder that typically affects the small joints in your hands and feet. Unlike the wear-and-tear damage of osteoarthritis, rheumatoid arthritis affects the lining of the joints, causing a painful swelling that can eventually result in bone erosion and joint deformity.

In 1988, Ben-Chetrit identified a protein, previously unknown, linked to Sjogren’s.

An autoimmune disorder, rheumatoid arthritis occurs when your immune system mistakenly attacks your own body’s tissues. In addition to causing joint problems, rheumatoid arthritis sometimes can affect other organs of the body — such as the skin, eyes, lungs and blood vessels.

Although RA can occur at any age, like Sjogren’s, it usually appears after age 40 and is much more common in women. The treatment, noted Ben-Chetrit, focuses on controlling symptoms and preventing damage to the joints.

Lupus, meanwhile, is also a chronic autoimmune and inflammatory disorder that can affect joints, skin, kidneys, blood cells, brain, heart and lungs. Lupus can be difficult to diagnose because its signs and symptoms often mimic those of other ailments.

The most distinctive sign of lupus – a facial rash that resembles the wings of a butterfly or the facial coloring of a fox – unfolding across both cheeks.

There is no cure for either of the conditions, which, again, are much more frequent in women, but symptoms can be controlled.

“Lupus is more ‘exotic’ disease than Sjogren’s so it attracts more researchers and investment of funds by drug companies and foundations,” said Ben-Chetrit. “More is also taught about it in medical school,” he pointed out. Some medications that help lupus patients also relieve symptoms in patients with Sjogren’s syndrome.

“If you asked a woman with such autoimmune diseases, she would probably prefer to have Sjogren’s than lupus,” he speculated.

Other secondary conditions related to Sjogren’s are pancreatic problems, tiredness, weight loss and even seizures. The lungs, kidneys or liver can also be affected. Probably the most serious secondary disease connected to Sjogren’s is non-Hodgkin’s lymphoma, a cancer of the lymphatic systems for which Sjorgen’s patients are 44 times at higher risk than non-Sjorgen’s patients, but fortunately, this occurs in only a small percentage.

While a Sjogren’s cure does not seem to be on the horizon, the three professors believe that improved medications and procedures will make it more livable in the coming years.


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