New drugs are in development for the treatment of lupus, one of which is likely to be the first medication in more than 30 years approved by the U.S. Food and Drug Administration to treat the devastating autoimmune disease, doctors here say.

Lupus is a chronic ailment that strikes primarily women, causing the immune system to attack and destroy DNA proteins in cells. Until now, except for a malaria drug that has a number of unpleasant side effects, there have been no treatments approved in three decades for lupus patients to combat the debilitating disease, which can affect many different systems in the body, says Dr. David Sandoval, a rheumatologist at Spokane-based Rockwood Clinic PS.

"It can affect almost everything," Sandoval says of lupus.

The disease is made more difficult to manage by the lack of approved medications, he says. Most of the treatments in use currently by rheumatologists who treat lupus patients are cancer-fighting drugs. The lack of approved medications has meant seeking approval from insurance companies of treatments that can cost up to $30,000 for a series of infusions, Sandoval says. Obtaining such approvals can be a difficult task, he says.

Sandoval says treatment options could change if one drug currently under consideration for FDA approval is approved as a treatment for lupus. The FDA has said that drug, belimumab, which was developed by Human Genome Sciences Inc. and GlaxoSmith Kline, likely will be approved later this year or early next year, Sandoval says. It would be marketed under the name Benlysta.

The drug works by blocking action by one of two kinds of white cells in the human body, called B cells, which appear to play a large role in the disease, Sandoval says. In lupus, B cells trigger immune system responses to the DNA of the nucleus of the body's cells, also inciting attack by T cells. B cells are produced in a person's bone marrow, while T cells are produced in the thymus, an organ in the chest cavity. Lupus is a broad term that encompasses a number of different categories of conditions from such attacks.

Arthritis Northwest Rheumatology PLLC, of Spokane, currently is enrolling patients for a phase-two study on a second drug, being made by Genentech, that's being developed as a potential treatment for lupus, says Dr. Eric Mueller, a rheumatologist in the practice. A phase two study involves the use of medications on human subjects.

The study drug, named rontalizumab, is called an "interferon inhibitor," which is an antibody to the hormone interferon that's produced by different types of cells in the immune system and promotes inflammation. The center hopes to enroll eight people here for the study, but Mueller says that's difficult because participants have to be having severe symptoms, but no symptoms involving the kidney. Lupus sufferers can become severely ill when the disease strikes their kidneys.

Much research has been done to try to understand the causes of lupus, but currently research is focused on improving treatment for it. There is little understanding of what really causes it, or why it affects women much more frequently than men, Sandoval says.

"Genetics seems to be involved, but the disease isn't inherited," he says. It doesn't seem to be racially specific, though Sandoval says that when African-Americans get it they often are stricken much more severely than others, though the reason isn't clear yet.

For the majority of patients, the disease is easily diagnosed, Sandoval says.

The main symptoms include joint pain, fatigue, unexplained rashes, especially across the face, and lesions in the mouth. It can cause hair loss, called alopecia, when it attacks the skin on a person's head, and it can attack the central nervous system, causing paralysis or seizures. Patients who have lupus also have a significantly increased risk of cardiac disease, Mueller says.

The disease usually strikes people in their prime active working years, in the 20s to 40s, Mueller and Sandoval each say, making it that much more devastating.

Though most people now live for a long time with lupus, it is associated with a reduced life span, and people used to die from organ failure and other complications from the disease, although physicians largely have been able to stop that from happening now, Mueller says. A lot of kidney dialysis patients have problems with kidney function because of lupus, he says. If their major symptom is fatigue because of low kidney function and they don't have rashes or other more visible signs of the disease, it's often not diagnosed until it has progressed quite far, he says.

With regular and sustained treatment, people have fewer and less severe flare-ups of the disease, he says.

Many patients who are having a lupus flare-up are treated with medicine that must be infused a number of times to get the condition under control, then continue with lower doses of medication indefinitely. Most of the medicines are infused, which can take several hours at an infusion center. Rockwood Clinic's lupus patients generally get their infusion treatments at one of its two cancer treatment centers, and Arthritis Northwest gives infusions onsite at its clinic.

"The ultimate goal would be to reverse the process that started it," Mueller says, but research just hasn't found a way to do that, he says.

Treating the disease can cause other problems, Sandoval and Mueller say. Many of the treatments for the disease are immune system suppressants, and make people more vulnerable to illness and infection. Sinus and urinary infections are the most common such ailments, and when a person gets either type of infection, treatment for lupus must be stopped until the infection can be cleared up, Mueller says. Even treatments such as bone marrow transplants have been performed at times, but with little success compared to the risks, Mueller says.

Mueller says about 65 percent of people with lupus are photosensitive, meaning they must avoid being in the sun. Even a little passive exposure, like having your arm resting on the window of a car while driving, can trigger a chain of events in the skin that can lead to a lupus rash. Mueller and Sandoval say this is because ultraviolet light kills skin cells, exposing their nuclei and triggering the immune response that causes a lupus flare-up.

Though lupus can be quite painful, people who have it often describe feeling like they have the flu when their disease is active, Sandoval says. He recommends that lupus patients stay as active as they can and work to maintain a good diet and exercise regularly to help keep a balanced immune system.

Sandoval says he also advises his patients to quit smoking if they haven't done so because smoking makes symptoms much worse by reducing the oxygen supply to the muscles.

He says many lupus patients appear to have low vitamin D levels, which seems to be somehow related to a high immune response, but the reason for that hasn't been determined. Sandoval says he recommends that lupus patients make sure they maintain an appropriate level of vitamin D, which he contends is hard to get through diet alone, although he also cautions that too much vitamin D can be toxic. He says he often prescribes folic acid supplements to his patients to promote organ health.