Eosinophilic Granulomatosis with Polyangiitis

As a rheumatologist, I often see patients who have spent years managing what they thought were unrelated health issues, such as asthma, sinus infections, allergies, nerve pain, or unexplained fatigue. Sometimes, these symptoms are connected by a single underlying condition.

One example is Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome.

EGPA is a rare autoimmune disease that causes inflammation of small and medium-sized blood vessels. It belongs to a group of conditions called ANCA-associated vasculitis, where the immune system mistakenly attacks blood vessels and causes widespread inflammation.

What makes EGPA unique is the combination of asthma, high levels of eosinophils (a type of white blood cell), blood vessel inflammation and organ involvement.

Because EGPA can affect multiple parts of the body, symptoms often appear unrelated at first. A person may visit an asthma specialist, an ENT doctor, a neurologist, and eventually a rheumatologist before the full picture becomes clear. The good news is that early diagnosis and modern treatments have significantly improved outcomes.

What Is Eosinophilic Granulomatosis with Polyangiitis?

EGPA is a rare autoimmune form of vasculitis that causes inflammation of small blood vessels. Inflamed blood vessels may become narrowed, damaged, swollen and less effective at delivering blood flow. This inflammation can affect the lungs, sinuses, skin, nerves, heart, kidneys and digestive system.

What Makes EGPA Different From Other Vasculitides?

Asthma is one of the most common features of EGPA. Many patients develop asthma years before other symptoms appear.
Eosinophilia: eosinophils are white blood cells involved in allergic responses. In EGPA, eosinophil levels rise abnormally high and contribute to ongoing inflammation.
Vasculitis: the immune system causes inflammation of blood vessels, which can damage organs.

What Causes Eosinophilic Granulomatosis with Polyangiitis?

The exact cause of EGPA remains unknown. Researchers believe EGPA develops due to an abnormal immune response. Immune system dysfunction, genetic factors, environmental triggers and allergy pathways may all play a role. However, having asthma or allergies does not necessarily mean a person will develop EGPA.

Early Symptoms of Eosinophilic Granulomatosis with Polyangiitis

Symptoms can vary significantly between patients.

Asthma that appears or worsens in adulthood: increased wheezing, more frequent attacks and difficulty controlling symptoms.
Chronic sinus problems: congestion, sinus pressure, chronic infections, nasal polyps and reduced sense of smell.
High eosinophil levels on a blood test.
Fatigue, low energy and weakness.
Fever and general illness, including night sweats.
Skin changes: purple spots, red patches, skin nodules and rashes.
Numbness and tingling, burning pain or weakness from nerve involvement.
Muscle weakness, such as difficulty walking or lifting objects.
Joint pain, stiffness and swelling.
Shortness of breath, persistent cough and chest discomfort.
Heart problems, such as inflammation around the heart or reduced heart function, which require ongoing monitoring.
Kidney involvement, less common than in other ANCA-associated vasculitides, with blood or protein in the urine.

How Rheumatologists Diagnose EGPA

There is no single test that can confirm the disease. Diagnosis requires a comprehensive look at the patient’s clinical picture.

Medical history: when asthma began, recurring sinus problems, nerve symptoms, eosinophil levels and multiple organ involvement.
Physical examination for skin changes, nerve abnormalities, breathing problems and joint inflammation.
Blood tests: eosinophil count, ANCA testing (some, but not all, patients are positive) and inflammatory markers such as ESR and CRP.
Imaging: chest X-rays, CT scans and heart imaging.
Biopsy of skin, lung, nerve or other affected tissue.

Treatment Options for Eosinophilic Granulomatosis with Polyangiitis

Treatment depends on the severity of disease, the organs involved and the risk of complications. The main goals are to control inflammation, reduce eosinophil activity, protect organs and achieve remission.

Corticosteroids are commonly used to quickly control inflammation.
Immunosuppressive medications help suppress abnormal immune activity, depending on severity.
Biologic therapy: medications that target eosinophil-driven inflammation may help control disease activity and reduce the need for steroids.
Asthma management, including inhaled medications, allergy management and pulmonary follow-up.

Living With Eosinophilic Granulomatosis with Polyangiitis

A diagnosis of EGPA can feel overwhelming, as the disease often affects multiple body systems. However, advances in treatment have significantly improved patient outcomes. Many patients can control asthma symptoms, prevent organ damage, continue normal activities and maintain an active lifestyle. Long-term care typically involves regular check-ups with a rheumatologist and other specialists.

When Should You See a Rheumatologist?

Seek evaluation if you experience adult-onset asthma, persistent sinus problems, high eosinophil levels, numbness or tingling, unexplained rash, shortness of breath, fatigue with inflammatory symptoms, or multiple symptoms affecting different organs.

Frequently Asked Questions

Is Eosinophilic Granulomatosis with Polyangiitis an autoimmune disease?

Yes. EGPA is an autoimmune vasculitis in which the immune system attacks blood vessels, causing inflammation.

Is Churg-Strauss Syndrome the same as EGPA?

Yes. Churg-Strauss Syndrome is the former name for Eosinophilic Granulomatosis with Polyangiitis.

Can EGPA be cured?

There is currently no cure, but many patients can achieve remission with treatment.

Does everyone with EGPA have ANCA antibodies?

No. Some patients test positive for ANCA antibodies, while others do not.

What specialist treats EGPA?

A rheumatologist usually coordinates care and often works closely with pulmonologists, allergists, neurologists, and other specialists.

A Rheumatologist’s Perspective on Eosinophilic Granulomatosis with Polyangiitis

One of the biggest challenges with EGPA is recognizing that symptoms affecting different parts of the body may actually be linked. Asthma, sinus problems, nerve symptoms, skin changes, and fatigue may seem like separate issues. However, when these symptoms occur together—especially alongside elevated eosinophils—a thorough evaluation is essential.

The most important message I share with patients is that EGPA is a serious condition, but it’s also highly treatable when diagnosed early.

Eosinophilic Granulomatosis with Polyangiitis: Understanding This Rare Autoimmune Disease