This inflammation reduces or stops the flow of blood to organs in the body.
This article discusses the symptoms and causes of granulomatosis with polyangiitis.
It also addresses the diagnosis and treatment of the disorder.
Verywell / Jessica Olah
Verywell / Jessica Olah
What Causes Granulomatosis With Polyangiitis?
As with all autoimmune disorders, GPA is characterized by an immune system malfunctioning.
For unknown reasons, the body mistakenly identifies normal tissue in blood vessels as foreign.
The immune cells then surround the blood vessel cells and form a hardened nodule known as agranuloma.
GPA mainly affects small- to medium-sized blood vessels.
Granulomatosis With Polyangiitis Symptoms
The symptoms of GPA vary by the location of the vascular inflammation.
In early-stage disease, frequent sinusitis and bloody noses are the most common symptoms.
It is not uncommon, for example, for GPA to be misdiagnosed and treated as a respiratory infection.
Other tools may be needed to support a diagnosis, including a biopsy of the affected tissue.
Together, the combination of tests and symptoms may be enough to support a GPA diagnosis.
After initial treatment, corticosteroid dosages are usually reduced as the disease is brought under control.
In some cases, the drugs may be stopped altogether.
How long can you live with granulomatosis with polyangiitis?
Newerantibody-based therapiesand a penicillin-like derivative calledCellCept (mycophenolate mofetil)may further improve those results in the coming years.
Symptoms vary depending on the location of inflammation and progression of the condition.
Treatment involves a combination of high-dose corticosteroids and immune suppressive drugs.
The length of treatment depends on the severity of the disease.
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National Health Service.Granulomatosis with polyangiitis.
National Organization for Rare Diseases.Granulomatosis with polyangiitis.
2015 Feb;28(1):17-27. doi: 10.1007/s40620-014-0135-3
Penn Medicine.Granulomatosis with polyangiitis.
