Table of ContentsView AllTable of ContentsCausesSigns and SymptomsDiagnosisTreatmentWhat Is the Prognosis for GPA Autoimmune Disease?
Table of ContentsView All
View All
Table of Contents
Causes
Signs and Symptoms
Diagnosis
Treatment
What Is the Prognosis for GPA Autoimmune Disease?
Granulomatosiswithpolyangiitis(GPA), formerly known as Wegener’s granulomatosis, is a rareautoimmune disorderthat causes inflammation, swelling, and irritation in blood vessels and other tissues.
This article discusses the symptoms and causes of granulomatosis with polyangiitis. It also addresses the diagnosis and treatment of the disorder.
Verywell / Jessica Olah
What Causes Granulomatosis With Polyangiitis?
The formation of granulomas can lead to chronic inflammation in the affected blood vessels (a condition known asvasculitis). Over time, this can weaken the vessels and cause them to burst, usually at the site of the granuloma. It can also cause the blood vessels to harden and narrow, cutting off the blood supply to organs or other parts of the body.
GPA mainly affects small- to medium-sized blood vessels. While the ears, nose, sinuses, kidneys, and lungs are the main targets of attacks, GPA can affect any part of the body.
GPA affects men and women equally, primarily in their 40s and 50s, but it can occur at any age.It is considered an uncommon illness with an annual incidence of only around 20 cases per one million people.
Vasculitis: Treatment for Autoimmune Symptoms
Granulomatosis With Polyangiitis Symptoms
However, as the disease progresses, other, more serious symptoms may develop, including:
The generalized nature of these symptoms can often make the diagnosis difficult. It is not uncommon, for example, for GPA to be misdiagnosed and treated as a respiratory infection. It’s only when healthcare providers can find no evidence of a viral or bacterial cause that further investigations may be ordered, particularly when there is evidence of vasculitis.
Systemic Symptoms
As a systemic disease, GPA can cause injury to one or several organ systems at once. While the location of symptoms can vary, the underlying cause (vasculitis) can usually point the healthcare provider in the direction of an autoimmune diagnosis if multiple organs are involved.
Systemic symptoms of GPA may include:
How to Test for Granulomatosis With Polyangiitis
The diagnosis of GPA is typically only made after several, unrelated symptoms go unexplained for a long period of time. While there are blood tests available to identify the specificautoantibodiesassociated with the disease, the presence (or lack) of antibodies is not enough to confirm (or reject) a diagnosis.
Instead, diagnoses are made based on the combination of symptoms, lab tests, X-rays, and the results of a physical examination.
Similarly, a chest X-ray or CT scan can often reveal lung abnormalities in persons with otherwise normal lung function.
Together, the combination of tests and symptoms may be enough to support a GPA diagnosis.
Granulomatosis With Polyangiitis Treatment
Before the 1970s, granulomatosis with polyangiitis was considered almost universally fatal, most often due to respiratory failure oruremia(a condition involving abnormally high levels of waste products in the blood).
In recent years, the combination of high-dosecorticosteroidsandimmune suppressive drugshas proven effective in achieving remission in 75% of cases.
By actively reducing inflammation with corticosteroids and tempering the autoimmune response with immune suppressive drugs like cyclophosphamide, many persons with GPA can live long, healthy lives and remain in remission for 20 years or more.
After initial treatment, corticosteroid dosages are usually reduced as the disease is brought under control. In some cases, the drugs may be stopped altogether.
In persons with severe disease, other, more aggressive interventions may be needed, including:
Despite high remission rates, up to 50% of treated individuals will experience a relapse.Moreover, persons with GPA are at risk of long-term complications, including chronic kidney failure, hearing loss, and deafness. The best way to avoid these is to schedule regular checkups with your healthcare provider as well as routine blood and imaging tests.
How long can you live with granulomatosis with polyangiitis?An absence of kidney involvement is associated with a 100% five-year survival rate, compared with approximately 70% in individuals with kidney disease.Overall, the 10-year survival rate ranges from 75%-88%.
How long can you live with granulomatosis with polyangiitis?
An absence of kidney involvement is associated with a 100% five-year survival rate, compared with approximately 70% in individuals with kidney disease.Overall, the 10-year survival rate ranges from 75%-88%.
Newerantibody-based therapiesand a penicillin-like derivative calledCellCept (mycophenolate mofetil)may further improve those results in the coming years.
Summary
Granulomatosis with polyangiitis is a rare autoimmune disorder that causes inflammation, swelling, and irritation in blood vessels and other tissues. 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.
7 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Johns Hopkins Medicine.Granulomatosis with polyangiitis.National Health Service.Granulomatosis with polyangiitis.National Organization for Rare Diseases.Granulomatosis with polyangiitis.Geetha D, Kallenberg C, Stone JH, et al.Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol. 2015 Feb;28(1):17-27. doi: 10.1007/s40620-014-0135-3Penn Medicine.Granulomatosis with polyangiitis.Safari S, Alesaeidi S, Pakzad B, et al.Predictors of relapse in granulomatosis with polyangiitis: a multi-center study. Egypt Rheumatol Rehabil. 2022;49(1):59. doi: 10.1186/s43166-022-00160-yMedscape.Granulomatosis with polyangiitis (GPA, formerly Wegener Granulomatosis).Additional ReadingAlmouhawis, H.; Leao, J.; Fedele, S. and Porter, S. “Wegener’s granulomatosis: a review of clinical features and an update in diagnosis and treatment.“Journal Oral Path Medicine.2013; 42: 507-516.Fortin, P.; Tejani, A.; Bassett, K.; and Musini, V. “Intravenous immunoglobulinin addition to standard treatments for Wegener’s granulomatosis.“Cochrane Data Syst Rev.2013; 1: DOI: 10.1002/14651858.CD007057.pub3Silva, S.; Specks, U.; Kaira, S. et al. ““Mycophenolate Mofetil for Induction and Maintenance of Remissionin Microscopic Polyangiitis with Mild to Moderate Renal Involvement—A Prospective, Open-Label Pilot Trial.“Clin J Am Soc Nephrol.2010; 5(3): 445-453.
7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Johns Hopkins Medicine.Granulomatosis with polyangiitis.National Health Service.Granulomatosis with polyangiitis.National Organization for Rare Diseases.Granulomatosis with polyangiitis.Geetha D, Kallenberg C, Stone JH, et al.Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol. 2015 Feb;28(1):17-27. doi: 10.1007/s40620-014-0135-3Penn Medicine.Granulomatosis with polyangiitis.Safari S, Alesaeidi S, Pakzad B, et al.Predictors of relapse in granulomatosis with polyangiitis: a multi-center study. Egypt Rheumatol Rehabil. 2022;49(1):59. doi: 10.1186/s43166-022-00160-yMedscape.Granulomatosis with polyangiitis (GPA, formerly Wegener Granulomatosis).Additional ReadingAlmouhawis, H.; Leao, J.; Fedele, S. and Porter, S. “Wegener’s granulomatosis: a review of clinical features and an update in diagnosis and treatment.“Journal Oral Path Medicine.2013; 42: 507-516.Fortin, P.; Tejani, A.; Bassett, K.; and Musini, V. “Intravenous immunoglobulinin addition to standard treatments for Wegener’s granulomatosis.“Cochrane Data Syst Rev.2013; 1: DOI: 10.1002/14651858.CD007057.pub3Silva, S.; Specks, U.; Kaira, S. et al. ““Mycophenolate Mofetil for Induction and Maintenance of Remissionin Microscopic Polyangiitis with Mild to Moderate Renal Involvement—A Prospective, Open-Label Pilot Trial.“Clin J Am Soc Nephrol.2010; 5(3): 445-453.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Johns Hopkins Medicine.Granulomatosis with polyangiitis.National Health Service.Granulomatosis with polyangiitis.National Organization for Rare Diseases.Granulomatosis with polyangiitis.Geetha D, Kallenberg C, Stone JH, et al.Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol. 2015 Feb;28(1):17-27. doi: 10.1007/s40620-014-0135-3Penn Medicine.Granulomatosis with polyangiitis.Safari S, Alesaeidi S, Pakzad B, et al.Predictors of relapse in granulomatosis with polyangiitis: a multi-center study. Egypt Rheumatol Rehabil. 2022;49(1):59. doi: 10.1186/s43166-022-00160-yMedscape.Granulomatosis with polyangiitis (GPA, formerly Wegener Granulomatosis).
Johns Hopkins Medicine.Granulomatosis with polyangiitis.
National Health Service.Granulomatosis with polyangiitis.
National Organization for Rare Diseases.Granulomatosis with polyangiitis.
Geetha D, Kallenberg C, Stone JH, et al.Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol. 2015 Feb;28(1):17-27. doi: 10.1007/s40620-014-0135-3
Penn Medicine.Granulomatosis with polyangiitis.
Safari S, Alesaeidi S, Pakzad B, et al.Predictors of relapse in granulomatosis with polyangiitis: a multi-center study. Egypt Rheumatol Rehabil. 2022;49(1):59. doi: 10.1186/s43166-022-00160-y
Medscape.Granulomatosis with polyangiitis (GPA, formerly Wegener Granulomatosis).
Almouhawis, H.; Leao, J.; Fedele, S. and Porter, S. “Wegener’s granulomatosis: a review of clinical features and an update in diagnosis and treatment.“Journal Oral Path Medicine.2013; 42: 507-516.Fortin, P.; Tejani, A.; Bassett, K.; and Musini, V. “Intravenous immunoglobulinin addition to standard treatments for Wegener’s granulomatosis.“Cochrane Data Syst Rev.2013; 1: DOI: 10.1002/14651858.CD007057.pub3Silva, S.; Specks, U.; Kaira, S. et al. ““Mycophenolate Mofetil for Induction and Maintenance of Remissionin Microscopic Polyangiitis with Mild to Moderate Renal Involvement—A Prospective, Open-Label Pilot Trial.“Clin J Am Soc Nephrol.2010; 5(3): 445-453.
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