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Overview

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare autoimmune condition. In EGPA, the immune system mistakenly attacks small- and medium-sized blood vessels, causing inflammation (vasculitis). This can reduce blood flow and damage organs and tissues. EGPA almost always involves the respiratory system and features high numbers of eosinophils, a type of white blood cell involved in allergic responses and fighting parasites.

EGPA is part of a group of diseases called ANCA-associated vasculitides, though not everyone with EGPA tests positive for ANCA (antineutrophil cytoplasmic antibodies). It typically develops in stages and can range from mild to life-threatening if organs like the heart, lungs, or nerves are severely affected. With modern treatments, many people achieve long-term control or remission.

Signs & Symptoms

Symptoms often appear in phases and vary widely depending on which organs are involved. Many people first have long-standing asthma or allergic rhinitis (hay fever) that becomes harder to control.

• Asthma that worsens or is difficult to manage, often with wheezing, shortness of breath, or coughing
• Chronic sinusitis, nasal polyps, or frequent sinus infections
• Allergic rhinitis or other allergy-like symptoms
• Fever, fatigue, night sweats, or unexplained weight loss
• Skin rashes, purpura (purple spots), or nodules
• Nerve problems (peripheral neuropathy) causing numbness, tingling, burning pain, or weakness in hands and feet
• Muscle or joint aches
• Abdominal pain, diarrhea, or bleeding if the gut is involved
• Chest pain, irregular heartbeat, or heart failure signs if the heart is affected
• Kidney involvement (less common than in other vasculitides), which may cause high blood pressure or changes in urine

Causes & Risk Factors

The exact cause of EGPA is unknown. It is an autoimmune process in which the immune system becomes overactive, producing excess eosinophils and attacking blood vessel walls. Genetics, environmental triggers (such as infections or allergens), and immune dysregulation likely play roles. Some cases have been linked to certain asthma medications (leukotriene modifiers) in people who already have underlying predisposition, but these drugs do not cause EGPA in most users.

• History of asthma or allergic conditions (nearly always present)
• Adult onset (most common between ages 30–50, though it can occur at any age)
• Possible genetic susceptibility
• No strong link to smoking or specific infections as primary causes

EGPA is rare, affecting roughly a few people per million each year. It is not contagious and is not directly inherited in a simple pattern.

Diagnosis

There is no single test for EGPA. Doctors diagnose it by combining clinical history, physical exam, lab tests, imaging, and sometimes biopsy. Key clues include asthma, eosinophilia (high eosinophil count in blood), and evidence of vasculitis in one or more organs.

• Blood tests: complete blood count (looking for high eosinophils), inflammatory markers (ESR, CRP), ANCA testing (positive in about 30–40% of cases, often p-ANCA/MPO), kidney and liver function
• Imaging: chest X-ray or CT for lung involvement, sinus CT, echocardiogram for heart
• Nerve studies (EMG/NCS) if neuropathy is suspected
• Biopsy of affected tissue (skin, lung, nerve, or other) showing eosinophilic inflammation and vasculitis when safe and needed
• Exclusion of other causes of eosinophilia (parasites, drug reactions, other hypereosinophilic syndromes)

Classification criteria (such as those from the American College of Rheumatology or newer ACR/EULAR criteria) help confirm the diagnosis in research and clinical settings. Early referral to a rheumatologist or vasculitis specialist is important.

Treatment & Management

Treatment aims to stop vessel inflammation, lower eosinophil counts, protect organs, and achieve remission while minimizing side effects. Therapy is tailored to disease severity and organ involvement.

• Glucocorticoids (steroids such as prednisone) are the foundation for most patients to quickly reduce inflammation
• Immunosuppressive or biologic medicines: for moderate-to-severe disease, drugs such as cyclophosphamide, azathioprine, methotrexate, mycophenolate, or rituximab may be used; mepolizumab (an anti-IL-5 antibody that targets eosinophils) is approved specifically for EGPA and helps many patients reduce steroid doses
• Asthma and allergy management: inhaled corticosteroids, bronchodilators, and sometimes additional biologics
• Supportive care: treatment of infections, blood pressure control, pain management for neuropathy, physical therapy
• Monitoring: regular blood tests, imaging, and specialist visits to adjust therapy and watch for flares or side effects

Many people reach remission (no active disease) and can taper steroids. Relapses can occur, so long-term follow-up is essential. Treatment decisions balance disease control with risks of infection and other medication effects.

Living With It

Living with EGPA means partnering closely with a care team (often including rheumatology, pulmonology, allergy/immunology, and primary care). Most people can lead active lives once the disease is controlled. Focus on medication adherence, infection prevention, healthy lifestyle habits, and recognizing early signs of a flare (worsening asthma, new nerve symptoms, fever, or fatigue).

Emotional support matters. Chronic illness can bring anxiety or frustration; counseling, support groups, and patient organizations can help. Vaccinations (as recommended by your doctor, avoiding live vaccines while immunosuppressed), bone health protection while on steroids, and regular exercise adapted to your energy levels all support long-term well-being.