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Patient and doctor go fishing.
Patient and doctor go fishing.


A Blood Vessel Disease that Can Be Lethal

Retired Baltimore policeman Tom Manzari is, well, hooked when it comes to fishing. So, last season, when his fingertips became too painful to manipulate the bait, he knew something was wrong.

The bizarre problem was just one in a series of ailments that had plagued Manzari. Years earlier, he’d been told he had a “deviated” nasal septum. Then he developed an unexplained hearing loss that doctors postulated might be due to a series of infections. Finally, when his aching fingertips began turning black, he was referred to the Rheumatology Division at Johns Hopkins. Within minutes, John Stone, M.D., M.P.H., recognized that this patient had not a deviated but a perforated nasal septum and diagnosed Wegener’s granulomatosis, a form of vasculitis.

Manzari was in the right place at the right time. Vasculitis—literally inflammation of the blood vessels—is awfully easy to miss, says rheumatologist David Hellmann, M.D., who with Stone has established a center to study and deal with the unusual disease. Left untreated, the linings of the injured blood vessels narrow or completely close. Blood flow (thus oxygen) is interrupted, and vital body tissue is damaged or destroyed.

The most common form of vasculitis, giant cell arteritis, affects the extracranial branches of the carotid artery, causing potential blindness. It strikes only about 25 in 100,000 mostly older adults annually, however, so people experiencing the telltale hearing, vision or breathing problems and severe head and jaw pain can find themselves wandering from doctor to doctor when their symptoms don’t respond to normal treatment.

Some 350 cases of vasculitis in the last three years have ended up at Johns Hopkins, where the new Vasculitis Center participates in an international network to combat the disease. The network currently is evaluating a new drug for giant cell arteritis and also studying whether another medication, Enbrel, can prove as effective in treating Wegener’s granulomatosis as it has in rheumatoid arthritis.

The cause of most forms of vasculitis remains unknown, Stone says. “One camp lumps it with autoimmune diseases and another believes it’s caused by some unknown infection.” As recently as 25 years ago, however, most patients died within two years of onset. Today, immunosuppressive and cytotoxic agents provide effective damage control, if not a cure. But Stone makes clear that patients require ongoing management by a specialist. Manzari lost two fingertips before his condition was controlled, but he’s back fishing, and recently even took Stone along. “The only fish the doc caught had already committed suicide,” he quipped.