Granulomatosis with polyangiitis (GPA): Symptoms, causes, and treatment

Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is a rare and life-threatening disorder that restricts the blood flow to several organs, including the lungs, kidneys, and upper respiratory tract.

It is a type of vasculitis, an inflammation and injury to the blood vessels.

GPA requires long-term immunosuppression. Some patients die due to the toxicity of the treatment.

GPA affects only 3 out of every 100,000 people in the United States.

It can develop at any age, but symptoms normally appear when patients are in their forties or fifties. It affects males and females are equally, and white people seem to be more susceptible than other ethnic groups.

As well as causing inflammation to the blood vessels, GPA produces granulomas around the blood vessels. A granuloma, or granulation, is a kind of localized nodular inflammation found in tissues, a type of inflammatory tissue.

The cause is unknown, but GPA is not a type of cancer or infection.

With early treatment and diagnosis, the symptoms of GPA can go into remission. Untreated, it can lead to kidney failure and death.

What is it?

[wegeners causes inflammation of the blood vessels]
GPA involves inflammation of the blood vessels.

GPA is part of a larger group of vasculitic syndromes.

They all feature an autoimmune attack by an abnormal type of circulating antibody called antineutrophil cytoplasmic antibodies (ANCAs).

These antibodies attack small and medium-sized blood vessels.

Apart from GPA, other examples of vasculitic syndromes include:

Churg-Strauss syndrome

microscopic polyangiitis

According to a description issued by the 2012 Chapel Hill Consensus Conference (CHCC2012), GPA is a:

“Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels (e.g., capillaries, venules, arterioles, arteries, and veins). Necrotizing glomerulonephritis is common.”

CHCC2012 also agreed to rename Wegener’s granulomatosis as granulomatosis with polyangiitis (GPA).

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Symptoms

Signs and symptoms of GPA can develop either gradually or suddenly.

The first symptom in most patients is rhinitis, a runny and stuffy nose, similar to a severe case of common cold.

Other signs and symptoms may include:

persistent runny nose

cough, which may include blood

ear infections

joint aches

loss of appetite

malaise, or a general feeling of being unwell

nosebleeds

shortness of breath

sinusitis

skin sores

swelling of joints

eye pain

burning sensation in the eyes

eye redness

vision problems

fever

weakness, often due to anemia

unintended weight loss

blood in the urine

It can lead to a rapidly progressive glomerulonephritis, a syndrome of the kidney that, if left untreated, rapidly progresses into acute renal, or kidney, failure. About 75 percent of people with the condition go on to develop kidney disease.

Causes

Experts do not know what causes GPA.

It appears to develop when an initial inflammation-causing event provokes an abnormal immune system reaction. This leads to inflamed and constricted blood vessels and granulomas, or inflammatory tissue masses.

When a relapse occurs, it is sometimes due to an infection.

Other contributory factors may be environmental toxins, a genetic predisposition, or a combination of both.

It has been linked to parvovirus and to a long-term presence of Staphylococcus aureus (S. aureus) in the nasal passage.

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