It often starts out as an uncomfortable feeling in the eyes and mouth. Patients say their eyes are dry and red, even though they are using eye drops. Often too, they say, their mouths are dry. Food is becoming tasteless. Some get swollen glands in their necks, making it look like they have mumps.
It turns out those are the hallmark clinical signs of Sjogren’s syndrome, a mysterious disease caused by an overproduction of B lymphocytes, the cells of the immune system that make antibodies. The deluge of B cells clogs glands. Some people have trouble perspiring because their sweat glands are obstructed. Or they have trouble digesting food. Women may have pain during intercourse because their vaginas become dry.
Sometimes, said Dr. Michael D. Lockshin, a rheumatologist and director of the Barbara Volcker Center at the Hospital for Special Surgery in New York, “you can bend the eyelid down and see lumps, collections of lymphocytes, that make the eyelids look pebbly on the inside.”
When Venus Williams said the disease made her feel tired, she was right. Patients with Sjogren’s, like those with the related diseases rheumatoid arthritis and lupus, are unusually tired, and there is no way to alleviate this sensation. Investigators have studied lupus patients, asking how much oxygen they consume when they exercise, and found that they use much more than healthy people, although no one knows why that is so.
It is hard to know how many people have Sjogren’s, because different doctors use different criteria for diagnosis. The hallmark of the disease is the presence of two unusual antibodies among the thousands produced, Ro and La, named with the first two letters of the last names of the initial patients in whom the disease was described decades ago. Some doctors diagnose Sjogren’s in anyone whose blood test shows Ro and La. But a third of patients with lupus, a similar disorder, and an unknown number of patients with rheumatoid arthritis also have Ro and La antibodies.
Other doctors only diagnose Sjogren’s if patients have Ro and La, dry eyes and mouths, and swollen salivary glands, but do not have signs of lupus or any other disease.
Prednisone, which suppresses the immune system, can offer immediate short-term relief of symptoms. People who are disfigured by swollen glands in their neck can look perfectly normal within 24 hours. But it cannot be taken for long periods because it has serious side effects, like osteoporosis, cataracts, facial swelling, weight gain, muscle wasting, early atherosclerosis and diabetes.
The simplest and safest treatment is the anti-malarial drug hydrocholor quinoline. No one knows why it slows the disease — its effects were discovered by accident when troops took it to ward off malaria. Some who had rheumatoid arthritis or lupus noticed that their symptoms were relieved.
But it can damage the retina over time and its effects are irreversible — the result is loss of vision.
So doctors are left trying drugs that suppress the immune system. But, Lockshin said, it is hard to know what will happen in the long run with those drugs, which were not tested in patients with Sjogren’s.
The long-term outlook for patients with Sjogren’s is not good. They are at increased risk of lymphomas, cancers of B cells. They are at risk for kidney disease, a scarring of the kidneys that can destroy their function, requiring patients to go on dialysis. And their dry eyes and dry mouths tend to get worse. Dry mouths lead to dental cavities and deterioration of the tissues of the mouth so that eating becomes a misery. Dry eyes after a while cannot be helped by eye drops.
It is not an encouraging picture, Lockshin admits. The disease has so many unknowns that, to a large extent, doctors’ hands are tied.
Sunday, September 4, 2011
The NY Times:
Posted by BA Haller at 10:10 AM