One of the first things you have to get to grips with if you or a family member has been diagnosed with this rare disease is what to call it! The most common name used out there, and the one most people would recognise is Wegener's Granulomatosis. It was named Wegener's after the doctor who first described the disease back in 1936, Friedrich Wegener.
In 2011 the name was formally changed from Wegener's Granulomatosis to Granulomatosis with Polyangiitis. You may also know it simply as Wegener's Disease, Wegener's Vasculitis, ANCA-associated Granulomatous Vasculitis, Weggies, WG or GPA.
Why was the name officially changed in 2011? Many diseases are named after the doctor who discovered and publicised the disease or it's symptoms, such as Alzheimer's disease. So why would we choose to dishonour Dr Wegener after all these years?
The answer goes back to the year 2000 when the medical journal The Lancet set out to write a piece celebrating Wegener. In the course of their research Dr. Eric Matteson, a rheumatologist at the Mayo Clinic, and Dr. Alexander Woywodt, a kidney specialist discovered that Wegener had a secret Nazi past, which he had hidden after the end of World War II.
Before the start of the war many doctors were forced to join the Nazi party to continue to practice medicine. Not so Wegener; he joined way back in 1932, even before Hitler had risen to power. He rose through the ranks as a military doctor and spent at least part of the war on just a few streets away from the Jewish Ghetto in Lodz, Poland.
The American College of Chest Physicians gave Wegener an award for his achievements in 1989 which was withdrawn in 2000 when his secret past became known. A campaign started to change the name to something other than Wegener's Granulomatosis. Understandably many people were upset by the name's Nazi connotations.
The question was which alternative name should we use, then?
For a while the front-runner was ANCA-associated Granulomatous Vasculitis, which is quite a mouthful.
In 2011 the Boards of Directors of the American College of Rheumatology (ACR), American Society of Nephrology (ASN) and the European League Against Rheumatism (EULAR), published an editorial and proposed the name Granulomatosis with Polyangiitis. It's not much less of a mouthful, but it can be shortened to GPA.
The name change is slowly percolating through the medical community and out into the wider community but it will be a long time before everyone can agree on the name.
In our house we still use Wegener's (pronounced vey-guh-nuh-s by the way).
What name do you use?
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