Vasculitis: The rare disease that killed Harold Ramis

Recently Dr. Jon Segal made an appearance on the Balancing Act addressing the topic of Rare Diseases and mentioning that Discount Medical Supplies is a supporter of though our charity program.  Global Genes is an organization dedicated to the research and awareness of rare diseases.  One of those rare diseases is Vasculitis, and it precisely the same one that took Harold Ramis' life.

Vasculitis is such a rare disease that in the entirety of Wikipedia there are only two deaths from this condition, and they are both Hollywood celebrities: Janet Leigh and Harold Ramis. It’s very safe to say that vasculitis was the only thing these two could ever have in common. Leigh is best known as the star of dramas and thrillers such as Orson Welles’ Touch of Evil, Alfred Hitchcock’s Psycho, and John Frankenheimer’s The Manchurian Candidate, while Ramis was the comic genius behind (and sometimes in front of) the camera in such satirical gems as Caddyshack, National Lampoon’s Vacation, and Groundhog Day. He also wrote and starred in Ghostbusters I and II as Dr. Egon Spengler, along with fellow funny men extraordinaire Bill Murray and Dan Aykroyd.

However, the dissimilarity between those two thespians comes to show that as rare a disease as vasculitis is, it certainly does not discriminate. As a matter of fact, even though some groups are more likely to develop it –namely people who have chronic hepatitis B or C infection, lupus, rheumatoid arthritis, and sclerodema, among other medical conditions and autoimmune diseases-, the truth is that this disorder can affect anyone, whether or not they have a Golden Globe. Vasculitis causes the immune system to attack blood vessel cells as if they were intruding viruses and bacteria, and symptoms include     fever, fatigue, weight loss, muscle and joint pain, loss of appetite, and nerve problems like numbness or weakness.

There are other symptoms that are more specific to some forms of vasculitis, such as Behcet’s syndrome (inflammation of arteries and veins; mouth and genital ulcers), Buerger’s disease (inflammation and clots in blood vessels in extremities; hand, arm, feet and leg pain, finger and toe ulcers), Churg-Strauss syndrome (affects blood vessels in the lungs), Cryoglobulinemia (rash on lower extremities, arthritis, weakness and nerve damage), giant cell arteritis (inflammation of arteries in the head), Henoch-Schonlein purpura (inflammation of blood vessels in skin, joints, bowel, and kidneys; abdominal pain, blood in urine, joint pain), hypersensitivity vasculitis (red spots on skin), Kawasaki disease (fever, skin rash, and eye inflammation), microscopic polyangiitis (skin lesions, fever, weight loss, nerve damage), polyarteritis nodosa (purpura, skin ulcers, muscle and joint pain, kidney problems), Takayasu’s disease (numbness or cold in extremities, decreased or absent pulse, high blood pressure, headaches, visual disturbances), and granulomatosis with polyangiitis (blood vessel inflammation in nose, sinuses, throat, lung and kidneys; nasal stuffiness, chronic sinus infections, nosebleeds).

Vasculitis is diagnosed with a battery of tests including blood, urine, imaging, and x-ray tests, as well as biopsy. It’s important to diagnose as early as possible because this disease can lead to organ damage, and as we’ve seen, death. Treatment consists mostly of medications; steroids to manage inflammation, and drugs to regulate the immune system. The former comprise corticosteroid drugs like prednisone and methylprednisone, while the latter involve medications that kill cells that cause inflammation when corticosteroids prove unsuccessful. Examples of those meds would be azathioprine, cyclophosphamide, and rituximab.

It is also worth mentioning that even if treatment yields positive results, there is always a chance of vasculitis returning. For instance, Ramis experienced a relapse in 2011, even after he had to relearn how to walk. Vasculitis patients may require ongoing treatment, as the disorder may stay with them permanently. Unfortunately, since the cause of vasculitis is largely unknown there is no way to prevent it. Nevertheless, there are ways of coping with it; some of those methods are eating a healthy diet and exercising regularly. Additionally, patients can educate themselves on the disease and its treatment, so as to be prepared for the side effects of the drugs they are prescribed.

Related Read: Discount Medical Supplies raises awareness of rare diseases