“Sneaky” is the word one rheumatologist used to describe vasculitis, a condition with wide-ranging symptoms that temporarily left actor Ashton Kutcher unable to see or hear.
Kutcher said he was “lucky to be alive” and that it took him a year to recover, during an episode of National Geographic’s “Running Wild With Bear Grylls: The Challenge.”
What exactly is vasculitis?
Vasculitis is an inflammation in the body’s blood vessels and arteries. The disease can hit out of nowhere and affect the function of any organ, including the lungs, heart, kidneys, eyes, brain, intestines and skin, said John Magaldi, MD, chief of rheumatology at the Hartford HealthCare Bone & Joint Institute.
“The blood vessels feed the organs, so inflammation can cause a narrowing of the blood vessel that results in slowing of blood to the organs. This can cause them to be damaged or even stop working,” he said, noting that the autoimmune disease has no well-known or identifiable cause. “It has eluded medicine.”
Different forms, different effects
Different forms of vasculitis affect the large, medium or small arteries in the body. The larger the arteries, the more important the function of the organs they feed, Dr. Magaldi said.
“Wegener’s vasculitis affects the large arteries to the lungs, kidneys and sinuses. On the opposite end, small vessel vasculitis impacts the skin and tips of the fingers.”
While Kutcher has not revealed the type that he has, Dr. Magaldi said one of the few varieties affecting hearing is Cogan’s vasculitis.
“Vasculitis is rare in general, but those affecting the hearing are even rarer,” he said, noting that Hartford HealthCare clinicians treat several hundred cases of vasculitis in different forms each year.
No known triggers
While clinicians have been unable to pinpoint triggers for vasculitis, Dr. Magaldi said the good news is that treatments have come “a long way” in his almost 30 years in practice. At one time, steroids were the only effective way to treat the inflammation, but newer medications and biologics have had “great success.”
“They work on the cells to reduce the overactivity of the immune system itself,” he explained.
Like Kutcher, 80% of people treated for vasculitis will attain remission and stay that way, Dr. Magaldi said. The other 20% will cycle between remission and inflammatory relapses.
Clinicians in many specialties have recognized the condition in patients presenting with various other symptoms, said Dr. Magaldi.
“We’ll get calls from a colleague with a 30-year-old who had a stroke, or a cardiologist who saw something odd on an echocardiogram, or a pulmonologist who sees lesions on a patient’s lung,” Dr. Magaldi said. “All can be signs of inflammation due to vasculitis.”
“It’s important for early intervention because this is something that can really tear through the body very quickly.”