The Rare Disease that Killed a Comedic Legend
Photo by Justin Hoch
By Rhonda Alexander
Many knew Harold Ramis, but few know what killed him.
The celebrated Hollywood filmmaker and Chicagoan passed away Monday at the age of 69. The cause of death was from complications related to a condition known as autoimmune inflammatory vasculitis, a disease that Ramis suffered from in private.
“Autoimmune inflammatory vasculitis is a general term because most vasculitis is autoimmune based, though it can be caused by infection,” says Sunil John, MD,
board-certified rheumatologist at Advocate South Suburban Hospital in Hazel Crest.
“The blood vessels become inflamed; they don’t supply the part of the body that’s affected with nutrients,” says John Stone, MD, a rheumatology specialist at Massachusetts General Hospital in Boston. “In vasculitis, there is inflammation in the vessel wall, the blood vessels become narrow, and the vessels can even die, leading to a condition called, ischemia, which [in layman’s terms] means, ‘not enough blood.’”
It is often aggressive and organ threatening. Failure to diagnose autoimmune inflammatory vasculitis early enough can lead to severe tissue damage and death. The symptoms and the consequences of this condition depend on the location of the vessels involved; but the gastrointestinal system, kidneys, eyes, lungs, brain and peripheral nerves can be affected.
Stacy Ardoin, a rheumatologist at the Lupus and Vasculitis Clinic at The Ohio State University, in Columbus, says that the condition is challenging to diagnose because the symptoms are often vague and can mimic other conditions such as lupus, another autoimmune disease, cancer or an infection. Patients exhibit symptoms and receive a diagnosis to treat these other conditions because there is no suspicion of vasculitis.
“This disease can be pretty sneaky,” she says. “You might blame [the symptoms] on an infection [which was the situation with Ramis] or just getting run down.”
John says that one indicator that could signal a specialist to look for this condition is when a patient has an infection that isn’t responding to traditional therapies. “Usually, antibiotics may be used to treat an infection, but if the patient isn’t getting better, ultimately, a referral to a rheumatologist who specializes in evaluating people for these disorders would make the call,” he says.
The most definitive way to diagnose the condition is through biopsy of the affected body part. “If the skin is affected, [we will] do a skin biopsy; if the brain is affected, [then we will] do a brain biopsy,” says John.
Blood testing and imaging studies, such as MRIs or angiograms also help to evaluate the blood vessels for diagnosis.
David Glass, MD, is the medical director and vein surgeon at New York Vein Center in NY. He says, “A biopsy of the artery will reveal giant cells—massive white blood cells that gobble up the debris inside the arterial wall leading to an inflammatory process, which causes the body to produce more collagen in an attempt to repair the damage. This ultimately leads to an obstruction in the artery.”
If the disease is caught early enough in its progression, Ardoin says that it can be treated with therapy, and some patients can achieve remission. But a lot of factors come into play when determining the prognosis of those who suffer from this condition.
“It depends on how early the condition is diagnosed as well as the type of vasculitis and which organs are involved,” says John. He says that age, severity of the condition, other comorbidities, such as diabetes, heart disease and kidney disease, all factor into an individual prognosis.
Unfortunately, because of the vague and mimicked symptoms that can often prevent doctors from looking for this disease specifically, autoimmune inflammatory vasculitis could progress to an irreversible point.
“Very often, it’s diagnosed at late stages after significant damage has already occurred, making it difficult for treatments to help,” says Stone. “The treatments—though they’ve improved in recent years—are fairly toxic.”
The toxicity Stone refers to is the use of steroids, which can cause an increase in blood pressure. It also predisposes people to diabetes and makes it harder to control for those who already have diabetes.
Prednisone, the typical steroid used in treatment, is your best friend and your worst enemy at the same time, says Ardoin. “It can raise cholesterol, cause weight gain and increase the risk of developing an infection.”
The steroid therapy can also be instrumental in thinning of the bones, which can lead to osteoporosis and the risk of fractures. Sleeping problems, mood changes and cataracts are possible side effects that patients experience when taking steroids, in addition to a host of other complications.
“Corticosteroids shut down your adrenal glands, and you retain a lot of water— everything in medicine is a [trade-off],” says Glass.
The causes of vasculitis in general are not known, which leads some to assume that genetics [is] the culprit. “At this time, we don’t have a strong sense of it being genetic,” says Ardoin, “so it’s possible there’s a genetic component involved, but by no means [does] genetics explain the whole disease.”
She says that a general lack of awareness about autoimmune diseases and vasculitis is what really leads to a delay in diagnosis. “As more people become aware of autoimmune diseases, they help others become diagnosed sooner and get appropriate treatment.”
Perhaps now, as the world mourns the death of Harold Ramis—especially Chicago—that awareness will increase.
Ramis is best known for acting in films such as Stripes and Ghostbusters, which he also co-wrote. He co-wrote and directed classic comedies such as Caddyshack, Groundhog Day and Analyze This. He also directed the John Hughes-penned National Lampoon’s Vacation staring Chevy Chase. Ramis was one of the three screenwriters who gave us National Lampoon’s Animal House. He trained with The Second City and was the original head writer, and a performer, on “SCTV.” He referenced his work as underdog comedy saying that “We attack the winners.” His career embodies the zeitgeist of the later half of the 20th century.
For more information on autoimmune inflammatory vasculitis, visit the American College of Rheumatology here.
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