EGPA vs GPA: What Patients Need to Know about these Autoimmune Diseases

If you’re dealing with persistent symptoms like late-onset asthma, chronic sinus issues, unexplained fatigue, or nerve pain—and standard treatments aren’t helping—you might be facing more than a common condition. 

Two rare but important autoimmune diseases to be aware of are Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Granulomatosis with Polyangiitis (GPA). Although their names sound similar, they are distinct types of ANCA-associated vasculitis and affect the body in very different ways. This article breaks down the differences between EGPA and GPA, explains their symptoms, how they are treated, and how common they are, using language that’s meant to support and inform patients.

What Is Eosinophilic Granulomatosis with Polyangiitis (EGPA)?

EGPA, formerly known as Churg-Strauss Syndrome, is a rare autoimmune condition. It most often begins with asthma and allergies, and progresses to involve the blood vessels and organs as stated by the Genetic and Rare Diseases Information Center (GARD). Patients may struggle with respiratory issues, such as persistent cough and trouble breathing. A key feature of the condition is eosinophilia, which means high levels of a certain white blood cell called an eosinophil. These cells can cause inflammation that damages tissues and organs.

Most people with this disease experience a progression through three phases:

1. Allergic phase – asthma, nasal polyps, or sinus issues
2. Eosinophilic phase – increased eosinophils in the blood or tissues
3. Vasculitic phase – inflammation of the blood vessels affecting nerves, skin, lungs, and other organs

What Is Granulomatosis with Polyangiitis (GPA)?

GPA, formerly called Wegener’s Granulomatosis, is another type of ANCA-associated vasculitis. It often affects the sinuses, lungs, and kidneys according to Mayo Clinic. Unlike EGPA, this conditon usually does not involve asthma or high eosinophil levels. Instead, it causes granulomas, or clusters of inflammatory cells, to form in affected tissues.

Common early symptoms include:

• Persistent sinus infections
• Nasal ulcers or nosebleeds
• Cough, sometimes with blood
• Fatigue, weight loss
• Kidney problems like blood in the urine

Key Differences Between EGPA and GPA

Although both diseases involve inflamed blood vessels, they affect the body differently:

• Asthma and Allergies: Common in EGPA, rare in GPA
• Eosinophilia: Found in EGPA, not typical in GPA
• ANCA type: EGPA is more often MPO-ANCA positive, while GPA is usually PR3-ANCA positive
• Organs Affected: EGPA may affect the heart and nerves; GPA commonly affects kidneys and upper respiratory tract

How Are Those Two Vasculitis Diseases Treated?

Both conditions are treated with medications that suppress the immune system, but the specific approach may vary.

• Corticosteroids are often the first step in treatment for both.
• Immunosuppressive drugs may be used for more severe cases.
• Biologics are increasingly used to manage difficult cases and prevent relapses.

Treatment goals are to control inflammation, protect organs, and maintain remission. Regular follow-ups are important for monitoring side effects and adjusting medications.

Prevalence Worldwide

Both EGPA and GPA are rare, but GPA is more common.

• EGPA affects about 2 to 38 people per million. Its incidence (new cases each year) is estimated at 0.6 to 3.4 per million.
• GPA affects up to 200-400 people per million. Incidence varies between 0.2 and 1.4 per 100,000 annually.

GPA is more frequently diagnosed in European populations, while EGPA tends to appear in people with a history of asthma and allergies in middle adulthood.

Final Thoughts

Living with a rare autoimmune condition such as the mentioned diseases  can be challenging, but understanding the differences can help you manage your health more confidently. Both conditions are serious, but with the right treatment and support, many patients go on to lead full, active lives.

If you have breathing troubles or other unexplained symptoms that might be linked to EGPA and suspect having a rare disease, you can use our free Risk Check for Rare Diseases. Just fill out our Patient Form and we do the rest. Get your secured analysis today, powered by AI and reviewed by our team of medical experts. Early diagnosis and treatment can make a significant difference!

References:

1. NIH Genetic and Rare Diseases Information Center (GARD). “Eosinophilic Granulomatosis with Polyangiitis.” https://rarediseases.info.nih.gov/diseases/6111/eosinophilic-granulomatosis-with-polyangiitis
2. Mayo Clinic. “Granulomatosis with Polyangiitis.” https://www.mayoclinic.org/diseases-conditions/granulomatosis-with-polyangiitis/symptoms-causes/syc-20351088
3. Bell C, Blauer-Peterson C, and Mao J (2021). Burden of illness and costs associated with eosinophilic granulomatosis with polyangiitis: evidence from a managed care database in the United States, Journal of Managed Care & Specialty Pharmacy 2021 27:9, 1249-1259, https://doi.org/10.18553/jmcp.2021.21002.
4. Emmi, G., Bettiol, A., Gelain, E. et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 19, 378–393 (2023). https://doi.org/10.1038/s41584-023-00958-w
5. DynaMed. Granulomatosis With Polyangiitis (GPA) and Microscopic Polyangiitis (MPA). EBSCO Information Services. Accessed June 20, 2025. https://www.dynamed.com/condition/granulomatosis-with-polyangiitis-gpa-and-microscopic-polyangiitis-mpa