Dr. Susan Manzi: Unmasking The Great Impersonator
Lupus is one of 80 known autoimmune disorders, which affect the body's system of specialized cells and organs that defend against germs. Diagnosis is an art, not a science—with no single blood test available to make the diagnosis certain.
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When Curtis Nugent’s knees began to ache, the athletic 19-year-old shrugged: too much basketball, thought the freshman at Robert Morris University, assuming the joint pain would subside. He didn’t know it was the first shot in his body’s war with its immune system.
His organs began a seven-month free fall. His kidney function crashed, and he was rushed to UPMC Presbyterian Hospital in April 2005. The diagnosis astonished his family: It was systemic lupus erythematosus (SLE)—often referred to simply as lupus, an autoimmune disease that affects more than 1.5 million Americans.
Nugent had a highly unusual case of a complex disease within a little-understood field; more than 50 million Americans suffer from autoimmune diseases, including Crohn’s disease and multiple sclerosis. Lupus, in particular, presents itself in so many different ways—from heart problems to skin rashes and arthritis—making diagnosis difficult.
The disease is rare in men, who comprise only 10 percent of patients. It is far more common in young women, striking Hispanics, Asians and African-Americans in particular. Symptoms may subside and reoccur over decades, and there is no cure.
“Lupus affects a lot more people than people realize,” says Nugent, now a 24-year-old elementary school teacher in the Wilmington School District. “I was fortunate it was caught in time. I’ve heard other stories—people don’t catch it in time, then need transplants or dialysis. Lupus hides itself so well it looks like other diseases. It’s hard to diagnose.”
His early diagnosis came from Dr. Susan Manzi, one of a limited number of lupologists worldwide. To Manzi, chair of the Department of Medicine at West Penn Allegheny Health System and director of its Lupus Center of Excellence, the bewildering disease is not just a professional specialty—it’s a personal mission.
Patients Are 'Like Snowflakes'
Fifty-one-year-old Manzi chose her specialty shortly after graduation from the University of Pittsburgh Medical School while completing a residency in internal medicine at Duke University. “I’ve spent 23 years focused on this disease,” says the Wexford resident—first at the University of Pittsburgh Medical Center and for the past 14 months at West Penn Allegheny. “I couldn’t be more passionate.”
Lupus is often called “The Great Impersonator,” mimicking indicators of other illnesses. Diagnosis is an art, not a science—with no single blood test available to make the diagnosis certain. Treatment has relied on therapies borrowed from other diseases, such as chemotherapy and drugs used to prevent organ-transplant rejection. “We think of our patients as snowflakes,” says Manzi. “No two are alike.”
But Manzi and her colleagues are cheering the recent approval of the first new lupus drug to be developed since 1955. The team at the Allegheny Singer Research Institute, the research arm of West Penn Allegheny, helped design the trial for the new monoclonal antibody, dubbed Benlysta, in which her patients participated.
Benlysta suppresses a protein known as B-lymphocyte stimulator, or BLyS. Elevated levels of BLyS stimulate the body’s production of autoantibodies, which attack healthy tissues.
Manzi calls the resulting process “self-sabotage.” The research challenge, she says, is: “Why would that happen in an otherwise healthy 20-year-old and change her life? You have to know every aspect of medicine—brain, kidney, skin—to treat lupus. It’s a comprehensive disease that is best managed by a team of specialists.”
This article appears in the May 2011 issue of Pittsburgh Magazine
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