Filmmaker Harold Ramis, who died Monday at age 69, suffered from autoimmune inflammatory vasculitis, one of more than 15 rare autoimmune diseases that can often be difficult to diagnose. He died from complications of the condition.
Ramis battled complications from vasculitis for several years, according to United Talent Agency, which represented the actor/writer/director. The agency said he died at home in the Chicago suburbs, surrounded by family and friends.
It occurs when the body's own immune system attacks the blood vessels by mistake, according to the Vasculitis Foundation, a support and education group based in Kansas City, Mo. This may happen as the result of an infection, a medicine, or another disease or condition.
As a result, the tissues and organs supplied by affected blood vessels do not get adequate blood and oxygen, which can result in organ and tissue damage that can lead to death.
There is no known cause or cure for vasculitis, on which there is scant research, the foundation says.
Though some forms improve on their own, most require treatment. The length of treatment varies, with some people using medications for extended periods.
People of all ages and races and both sexes are at risk for vasculitis, according to the National Heart, Lung and Blood Institute. Some types of the condition seem to occur more often in people who:
• Have certain medical conditions, such as chronic hepatitis B or C infection
• Have certain autoimmune diseases, such as lupus, rheumatoid arthritis and scleroderma
Kawasaki disease, a type of vasculitis in which the walls of the blood vessels throughout the body become inflamed, exclusively affects children, says rheumatologist Peter Merkel, professor of medicine and epidemiology at the University of Pennsylvania and principal investigator of the Vasculitis Clinical Research Consortium.
"Vasculitis can sometimes look like other diseases, so it can be missed initially," he says.
The various types and symptoms of the disease "can range from a self-limited, one-time process to a chronic, lifelong recurrent disease, and it can range from minor symptoms to a very organ-threatening and life-threatening disease, and any combination thereof," Merkel adds.
The number of people affected "ranges greatly," he says. Most of the individual types "have a prevalence of fewer than 200,000 cases. But added all together, millions of people are affected."
Autoimmune inflammatory vasculitis, the condition that Ramis reportedly had, "is somewhat a redundant term," says Merkel, adding that not enough details have been reported to know the specific type that he had. "Sometimes we don't know the exact type."
In Ramis' case, "it sounds like he had a neurologic disease, which can be a very painful and serious problem, where the blood vessels supplying the nerves are damaged, so the nerves die. You can't move the hand or foot. There can be a lot of pain and dysfunction."
Treatment of severe vasculitis involves prescription medicines, including corticosteroids and cytotoxic medicines, according to the National Heart, Lung and Blood Institute. "Rarely, surgery may be done. People who have mild vasculitis may find relief with over-the-counter pain medicines, such as acetaminophen, aspirin, ibuprofen or naproxen."
"A lot of research is being done to try and reduce the burden of disease, and to try and get treatments that are less toxic, and we're making progress," Merkel says.