Disease Discovered like Mysterious AIDS

Researchers have named a mysterious new disease that has left marks of people in Asia and some in the United States with AIDS like symptoms yet though they are not infected with HIV.

The patients' immune systems become damaged, departure them unable to fend off germs as healthy people do. What triggers this is not known, but the disease does not appear to be contagious.

This is another kind of acquired immune lack that is not inherited and occurs in adults, but doesn't spread the way AIDS does done a virus, said Dr. Sarah Browne, a scientist at the National Institute of Allergy and Infectious Diseases.

She helped lead the study with investigators in Thailand and Taiwan, where most of the cases have been found since 2004. Their report is in Thursday's New England Journal of Medicine.

"This is absolutely fascinating. I've seen likely at least three patients in the last 10 years or so" who might have had this, said Dr. Dennis Maki, an infectious disease specialist at the University of Wisconsin in Madison.

It's still possible that an infection of some sort could trigger the disease, even though the disease itself does not appear to spread person to person, he said.

The disease develops around age 50 on averages, but does not run in families, which makes it unlikely that a single gene is responsible for, Browne said.

Some patients have died of overcoming infections, including some Asians now living in the U.S., although Browne could not estimate how many.

Kim Nguyen, 62, a seamstress from Vietnam who has experienced in Tennessee since 1975, was gravely ill when she attempted help for a persistent fever, infections throughout her bones and other bizarre symptoms in 2009. She had been crazy off and on for several years and had visited Vietnam in 1995 and again in early 2009.

"She was wasting away from this systemic infection" that at first appeared like tuberculosis but wasn't, said Dr. Carlton Hays Jr., a family physician at the Jackson Clinic in Jackson, Tenn. "She's a small woman to start with, but when I first saw her, her weight was 91 pounds, and she lost down to 69 pounds."

Nguyen was concerned to specialists at the National Institutes of Health who had been tracking similar cases. She exhausted nearly a year at an NIH hospital in Bethesda, Md., and is there now for monitoring and further treatment.

"I feel great now," she said Wednesday. But when she was sick, "I felt dizzy, headaches, nearly fell down," she said. "I could not eat anything."

AIDS is a specific disease, and it stands for acquired immune deficiency syndrome. That means the immune system becomes marred during someone's lifetime, rather than from inherited gene defects like the "bubble babies" who are born unable to fight off germs.

The virus that causes AIDS - HIV - destroys T cells, key soldiers of the immune system that fight germs. The new disease does not impress those cells, but causes a different kind of damage.

Browne's study of more than two hundreds people in Taiwan and Thailand detected that most of those with the disease make substances called auto antibodies that block interferon gamma, a chemical signal that helps the body clear infections.

Blocking that signal imparts people like those with AIDS - vulnerable to viruses, fungal infections and parasites, but especially micro-bacteria, a group of germs similar to tuberculosis that can cause severe lung damage. Researchers are calling this new disease an "adult-onset" immunodeficiency syndrome because it formulates later in life and they don't know why or how.

"Fundamentally, we do not know what is inducing them to make these antibodies," Browne said.

Antibiotics aren't always effective, so doctors have attempted a variety of other approaches, including a cancer drug that helps suppress production of antibodies. The disease quiets in some patients once the infections are tamed, but the faulty immune organization is likely a chronic condition, researchers conceive.

The fact that nearly all the patients so far have been Asian or Asian people living elsewhere proposes that genetic factors and something in the environment such as an infection may trigger the disease, researchers conclude.

The first cases turned up in 2004 and Browne's study recruited about one hundred people in six months.

"We know there are many others out there," including many events mistaken as tuberculosis in some countries, she said.