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Understanding Your GPA Diagnosis

You may still see the name Wegener’s granulomatosis in older medical records or online. In 2011, the medical community officially changed the name to Granulomatosis with polyangiitis. This change was made for two reasons: to use a name that better describes the disease (inflammation of vessels and granulomas) and to distance the condition from Dr. Friedrich Wegener, whose past association with the Nazi party was deemed incompatible with the values of the medical profession.

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 Granulomatosis with polyangiitis

GPA may not be the leading diagnosis initially. That's because GPA is rare and other, more common conditions (such as pneumonia or sinus infection) can cause many of the same symptoms. As a result, the average person with GPA often has symptoms for months before the correct diagnosis is made.

 He Was an Active Guy. Suddenly His Legs Ached After a Few Blocks

GPA is a disease of the small- and medium-size arteries in the body, which is a reason it can affect so many different parts. It is characterized by the development of granulomas — clumps of white cells and other tissues that form in reaction to infection or inflammation — in the lungs, in the upper respiratory tracts and in the kidneys. Untreated, the disease can kill, destroying the involved tissues.

Articles of Interest

A Nazi Past Casts a Pall on Name of a Disease

The movement to discredit the Wegener name is beginning to gather momentum. The chest physicians’ group has rescinded its prize. Dr. Matteson and others, including some of the tens of thousands of American patients with Wegener’s granulomatosis, seek a more descriptive term. At the moment, the most precise candidate is ANCA-associated granulomatous vasculitis, which does not exactly trip off the tongue.

At 54, Cindy Abbott Runs the Iditarod to Raise Awareness of Her Rare Disease

Some people with this disease can't even walk their dogs. The doctors would say I shouldn't be able to do this, but I kept climbing bigger mountains and nothing would happen.

Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA)...

This study represents the largest cohort of GPA reported to date. Pediatric GPA patients experienced more frequent hospitalizations and were more vulnerable to hematologic complications than non-elderly adult patients.

Eric Lynn: My journey with Granulomatosis with polyangiitis (GPA)

Nothing would prepare me for the journey that was waiting for me with this rare autoimmune disease, Granulomatosis with polyangiitis (Wegener’s). Months before my diagnosis, I was feeling horrible with muscle and joint pain, extreme fatigue, and what seemed like bad allergies. Then I had an unusual headache with a severe eye pain that was frightful. I could see my optometrist right away, who told me at that time that this was something systemic and pointed me in the right direction to a rheumatologist.

Living & Eventually Dying With Wegener's

If Wegener's Granulomatosis attacks just one in two million (caucasians only) then interested parties appear very limited. World wide that would only equate to 20,000 folks at best and probably many of those sickies do not read English. Hence, statistically I should probably forget this exercise in futility. However, while dying I vowed to write about my ordeal because there are millions of people who could contract said disease and if this article helped them or their physician to diagnose earlier, the hour or so I spend would be well spent.

My Journey with Wegeners Granulomatosis

A lot of people are curious to know about Wegeners Granulomatosis when they hear that I have it. I think it is important to get the word out about Vasculitis diseases because knowledge is power and the more people that know about Wegeners, the better chance we have to finding a cure.

“What is… Wegener’s Granulomatosis?”

Finally, the next generation of medical AI computers will ultimately “learn” from their experience. Once every patient’s data is stored in the computer and the final, correct answer is also captured by the system, the AI program need not rely only on textbook chapters and articles as its source of data. Instead, it could learn that patients like the one you are seeing ultimately turned out to have Wegener’s granulomatosis, even though they were frequently mistakenly diagnosed initially as having atypical pneumonia or sinusitis. And it could adjust its algorithm accordingly. This, of course, is analogous to Amazon.com’s magical feat of informing us that “customers like you bought X book.” Except it would be “patients like yours had Y disease.”

Resources

ANCA Pathways

Don't miss the role the complement pathway plays in ANCA-associated vasculitis (GPA/MPA)

ANCA-Associated Vasculitis

GPA is a disease resulting from inflammation of the blood vessels. In GPA, immune cells can cluster to form what is called a granuloma. The lungs, kidney, sinuses, ears, nose, and throat are often affected.

Rituxan

Rituxan with glucocorticoids is the only FDA-approved induction treatment for adults with Granulomatosis with Polyangiitis (GPA) (formerly known as Wegener's Granulomatosis) and Microscopic Polyangiitis (MPA)

Living with Wegener's Granulomatosis

During the summer of 2007, I was diagnosed with a rare autoimmune disease called Wegener's Granulomatosis. This is a chronicle of my life, symptoms and medical treatment.

Wegener's Disease

Everything you need to know about Wegener's Disease. Also known as Granulomatosis with Polyangiitis (GPA).

Wegeners Granulomatosis / GPA Support and Information

Welcome to the Wegeners Granulomatosis Disease Support Forums. We hope you find lots of valuable information here. There is information, support and friendly chat here for sufferers, their family and friends. We hope you sign up and become part of this very supportive group of people.

Weggieboy's Blog

Surviving retirement with two cats. It isn’t a catwalk, but one does need to focus on what IS possible, not the things lost.

Vasculitis Foundation

The medical community believes GPA/Wegener’s is not so rare, but rarely diagnosed. In areas where doctors are aware of the disease, more patients are diagnosed and treated. Early diagnosis and treatment are essential to improve patient outcomes and prevent organ failure.

Vasculitis UK

The name of this vasculitic disease was officially changed from Wegener's Granulomatosis (WG) to Granulomatosis with Polyangiitis (GPA) in 2011. Throughout this descriptive section it will be referred to as GPA.

Cleveland Clinic

Granulomatosis with polyangiitis (Wegener’s) (GPA) is a rare disease of uncertain cause. It is the result of inflammation within the tissues called granulomatous inflammation and blood vessel inflammation ("vasculitis"), which can damage organ systems. The areas most commonly affected by GPA include the sinuses, lungs, and kidneys, but any site can be affected.

Johns Hopkins

The first case was described by Heinz Klinger, a German medical student, in 1931. Several years later a German pathologist, Friedrich Wegener, described 3 additional cases and recognized the disorder as a distinct form of vasculitis. Other names occasionally used for Wegener’s Granulomatosus are Wegener’s arteritis or Wegener’s disease.

MayoClinic

Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs. Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious. Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue. There is no known cause for Wegener's granulomatosis.

MedicineNet

Wegener's granulomatosis is an uncommon type of inflammation of small arteries and veins (vasculitis). It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages(sinuses), and the kidneys. "Incomplete" forms exist that only involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized Wegener's granulomatosis.

NHS

It's a serious condition that can eventually lead to tissue damage and organ failure and can be fatal if left untreated. However, there are effective treatments which mean most people can live relatively normal lives with the condition.

NORD

Granulomatosis with polyangiitis (GPA) is a rare disorder characterized by inflammation of small- and medium-sized blood vessels (vasculitis) that results in damage to various organ systems of the body, most often the respiratory tract and kidneys.

Patient

Wegener's granulomatosis is a rare form of vasculitis. It is thought to be an autoimmune inflammatory process affecting endothelial cells. It is a multisystem disease which can affect many parts of the body, categorised by the ELK classification: it most commonly presents with lesions in the upper respiratory tract (E - indicating ears/nose/throat, almost 100%), lungs (L - most patients) and kidneys (K - >75%).

Rheumatology.org

Another recent option for the treatment of severe granulomatosis with polyangiitis is rituximab(Rituxan) combined with glucocorticoids. Rituximab is a biologic agent given by vein that targets a type of immune cell called the B lymphocyte.