All In A Name: How Wegener’s Granulomatosis Became Granulomatosis With Polyangiitis

Eponyms are very common in medical terminology — diseases, diagnostic and treatment procedures, and even surgical instruments are all often named after the people who discovered or invented them. The past few decades have, however, sparked heated debates about some eponyms. The disease granulomatosis with polyangiitis (GPA), which was previously called Wegener’s granulomatosis after German physician and pathologist Friederich Wegener, has been at the forefront of this controversy. 

Why was Wegener’s granulomatosis renamed to GPA?

The physician and pathologist Friederich Wegener who first noticed cases of patients with destructive granulomatosis in the upper respiratory tract published his papers describing this condition in 1939. They were reviewed after World War II, and the condition that first caught his attention was named after this German doctor — Wegener's granulomatosis. The ACCP even honored him as a Master Clinician in 1989.

By the year 2000, however, researchers made the startling finding that Wegener cooperated with the Nazis and even conducted medical experiments on prisoners in Poland during WWII. An initiative to change the official name of the disease was in full swing two years later, and by 2011, the medical community had come to a consensus to do away with the old eponym. The condition was now to be known as Granulomatosis With Polyangiitis.

The power of habit: Why you may still encounter the name Wegener's granulomatosis​

Although medical professionals are now obliged to use the new official name, GPA, many still talk about "Wegener’s granulomatosis", probably because they are uncertain that the consensus has reached everyone in the medical community and want to be sure that everyone understands what condition is being discussed.

The transition to the name GPA is definitely a positive thing, but it will take some time to fully replace the old term in everyone’s medical vocabulary.

What is GPA?

GPA is a relatively rare type of vasculitis, or inflammation of the blood vessels. It primarily affects small blood vessels in the walls of the upper and lower respiratory tract, as well as in the kidneys. By morphology, this inflammation is granulomatous — hence the word granulomatosis in the name. The cause of GPA is still unknown, but many factors suggest that it is an autoimmune disease. As studies indicate, genetic factors seem to have an influence, and one can inherit a predisposition for GPA. However, environmental factors are important triggers. This is the case with most autoimmune diseases.

How does GPA manifest?

GPA is a systemic vasculitis — meaning it can cause inflammation of small blood vessels in many different organs and tissues — but the most distinct are granulomatous lesions in the respiratory tract and glomerulonephritis (inflammation of the kidneys).

People with this disease may feel the signs of any systemic inflammatory disease, which include:

• Fatigue
• Fever
• Joint and muscle pain
• A general unwell feeling.

On the skin, GPA can cause rash, redness, and even bleeding ulcers. In other organs, its symptoms are also related to blood-vessel damage: coughing with bloody sputum, nose bleeds, and blood effusions in most tissues, including the brain and the spinal cord, liver, eye structures, and gastrointestinal system. This can lead to visual and hearing impairment, central and peripheral nervous system problems, blood in the stool, and other very serious symptoms.

The diagnosis of GPA is very difficult to make because there are no established diagnostic criteria. It comes down to physical examination, detection of auto-antibodies, and findings of vasculitis and necrosis in a biopsy of the affected organs.

How is GPA treated?

The medications used in the treatment of GPA are not much different than those prescribed to patients with any other autoimmune disease. Glucocorticoids reduce the autoimmune response and inflammation, and cyclophosphamide also strongly suppresses the intense autoimmune response which exists in this disease.

Since this condition affects the blood vessels of multiple organs, a multidisciplinary approach is required to assess how well the treatment is working and to try to repair the damage that was already done to certain organs.

Other controversial eponyms

The numerous other examples of inappriopriate eponyms that led to a general trend towards descriptive disease names include:

• Reactive arthritis. Formerly known as Reiter’s syndrome, it was named fter Hans Reiter, a German physician who was also involved in Nazi activities and medical experiments on prisoners during World War II. After the war, the eponym was replaced with the descriptive name reactive arthritis.
• Asperger syndrome. Named after Hans Asperger who was, again involved in Nazi activities, it was incorporated into the unified diagnosis of Autism Spectrum Disorder under the DSM-5.
• Spatz–Stiefler reaction. Named after Hugo Spatz and Georg Stiefler, also known for their associations with Nazi groups, this disorder is now commonly called paralysis agitans reaction.

While conditions named after Nazis are a kick in the face, not just to patients suffering from them but to the rest of humanity too, they are, fortunately, gradually disappearing from the medical vocabulary in favor of neutral and descriptive terms.