Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Diseases of the immune system Vasculitis

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Rare Disease Classifications:

ORPHA:183 Eosinophilic granulomatosis with polyangiitis

ICD-11:A44.A2 Eosinophilic granulomatosis with polyangiitis

IMPORTANT INFORMATION

The information on the Rare Awareness Rare Education (RARE) Portal is intended for educational purposes only and does not replace professional advice.

Rare diseases typically display a high level of symptom complexity and variability. Individuals diagnosed with the same rare disease may be impacted differently and each person’s experience is unique. Please seek support from qualified healthcare professionals to learn more about the most suitable care and support options for you.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.

Emergency Management

There may be special considerations for the emergency management of individuals living with eosinophilic granulomatosis with polyangiitis (EGPA) presenting to emergency departments.

Clinical Care Guidelines

We are not aware of any clinical care guidelines for eosinophilic granulomatosis with polyangiitis (EGPA) in Australia. If you know of any relevant care guidelines, please let us know via the Contribute page.

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

Synonyms

EGPA, CSS, Churg-Strauss vasculitis, allergic granulomatosis, allergic granulomatous angiitis, allergic angiitis.

Previously known as Churg-Strauss syndrome (CSS), it was renamed eosinophilic granulomatosis with polyangiitis (EGPA) in 2012.1

Summary

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterised by chronic asthma and inflammation of small and medium blood vessels.2-4 EGPA typically develops in three stages with distinct features. The ‘early phase’ involves asthma and hay fever which can occur years before the onset of any other symptoms. The ‘eosinophilic phase’ begins when a type of white blood cell, called ‘eosinophils’, are overproduced. Eosinophils usually fight pathogens (bacteria and viruses) but in EGPA, they build up in the blood stream and organs where they cause damage. As they accumulate, they may form clusters called granulomas which promote inflammation. The ‘vasculitic phase’ begins when vasculitis (sometimes called angiitis) starts. Vasculitis is the inflammation and narrowing of blood vessels. In EGPA, vasculitis affects many small and medium blood vessels throughout the body and prevents adequate blood flow to nearby body parts. The accumulation of eosinophils (eosinophilia) and vasculitis separately or together can cause serious tissue and organ damage. The affected body parts and associated complications differ between individuals depending upon where the vasculitis and inflammation occur. The three phases of EGPA development can occur at the same time, or years apart, and in any order.

Some individuals with EGPA have been found to have anti-myeloperoxidase (MPO)-anti neutrophil cytoplasmic antibodies (ANCA) in their blood. These auto-antibodies are not specific to EGPA but are present in a few different types of vasculitis conditions. The presence of these MPO-ANCA antibodies and how they may contribute to EGPA is still not well-understood.3, 5, 6

Personal Stories

Eosinophilic granulomatosis with polyangiitis (EGPA) varies between individuals, and each person’s experience is unique.

If you would like to share your personal story with Rare Voices Australia and have it included on the RARE Portal, please visit Rare Voices Australia: Share Your Story.

Symptoms

Symptoms of eosinophilic granulomatosis with polyangiitis (EGPA) vary widely between individuals and change as the disease progresses.1-5

Asthma is typically the first symptom of EGPA, sometimes occurring years before any other signs.1,3,6 It is usually severe and worsens over time (preexisting and progressive asthma). This may coincide with other allergy-like symptoms such as hay-fever (allergic rhinitis), painful sinuses or face (sinusitis) and runny or blocked nose. Small ball-like bumps can often be found inside the nose or sinus (nasal polyps) early in the disease. Some people also experience flu-like symptoms such as feeling generally unwell with aches, tiredness, fever, and swollen lymph nodes.

Symptoms typically progress to include signs of organ damage during the eosinophilic and vasculitic phases of EGPA .1-7 The symptoms and their severity depend upon which blood vessels and organs are affected. The symptoms of EGPA can be extremely broad and are not just limited to these characteristic symptoms listed below:

  • abdominal pain, nausea, stomach cramps and/or vomiting (gastritis, enteritis, or colitis)
  • skin rashes which may cause scarring
  • nerve damage (neuropathy) including pain or burning, numbness or tingling, weakness or loss of feeling anywhere in the body
  • kidney disease which may result in high blood pressure (renal hypertension)
  • pus, blood, small clumps of cells (polyps) or other unusual substances in the lungs (pulmonary infiltrates). The most common sign of this is pneumonia.
  • chest pain and/or heart palpitations

There may also be complications associated with EGPA, including life-threatening respiratory issues, kidney failure, blood clots (thrombosis), aneurysms, permanent loss of feeling in hands or feet, stroke, and heart failure.2-4, 6-8, 9

Please speak to your medical team to learn more about the symptoms and complications of EGPA.

Cause/Inheritance

The cause of eosinophilic granulomatosis with polyangiitis (EGPA) is not fully understood. It is thought that it may be caused by a combination of environmental factors (such as allergens and infections), an overactive immune system, and genetics.2,3,8

Diagnosis

Eosinophilic granulomatosis with polyangiitis (EGPA) may be diagnosed based on physical examination, extensive medical history, blood tests to check for high levels of eosinophils (a type of white blood cell) and MPO-ANCA antibodies, and imaging scans.5,6,8,9 Scans may include X-ray and computed tomography (CT) to detect for abnormal clumps of cells (polyps) in the sinuses and lungs.

More information about the blood tests can be found at

A tissue sample (biopsy) may also be taken to look for inflammation and narrowing of blood vessels (vasculitis/angiitis) and the presence of eosinophils (white blood cells). The presence of vasculitis and eosinophils can help to distinguish EGPA from other diseases that have similar signs and symptoms. Other tests, such as urine tests and heart scans are commonly done to look for signs of organ damage and/or assess the extent of complications.

Recommendations for diagnosis of EGPA that were developed by a panel of European experts can be found at Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.

Differential diagnosis (to rule out other conditions) includes eosinophilic lung diseases (such as eosinophilic pneumonia, allergic bronchopulmonary aspergillosis), other types of small and medium vessel vasculitis (such as granulomatosis with polyangiitis, polyarteritis nodosa) fungal and parasitic infections, and malignancy.2-4,6,7

Please speak to your medical team to learn more about the available diagnostic pathways for EGPA.

Treatment

There is no curative treatment for eosinophilic granulomatosis with polyangiitis (EGPA), but there are management strategies to control eosinophil levels and inflammation, which may involve:4,6,8,10

  • corticosteroid medication to reduce inflammation
  • medication that suppresses the immune system (immunosuppressive drugs)
  • biologic medications which inhibit certain inflammatory pathways in the body

As individuals with EGPA are at risk of life-threatening heart and kidney complications, it is recommended that their heart and kidney health are regularly monitored to allow for early detection and interventions to manage such complications.2,4 It has also been recommended that individuals with asthma avoid smoking and second-hand smoke.10,11

There are also management strategies for treating the other complications associated with EGPA, such as chronic asthma, kidney disease, nerve damage and skin damage.6 It is best to speak to speak with your medical team to learn more about the different types of management strategies for EGPA and its associated complications. Treatment will depend on an individual’s specific symptoms and the affected organ systems.

Clinical Care

Healthcare professionals involved in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) may include general practitioners (GP), paediatricians, immunologists, cardiologists, dermatologists, otolaryngologist, rheumatologist, pulmonologist.12,13 The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

Research and Data

ausEE Inc.: Research  provides information about research on eosinophilic diseases in Australia, including funding for medical research grants.

Please visit Australian Clinical Trials to learn about clinical trials for eosinophilic granulomatosis with polyangiitis (EGPA) in Australia; there may not be any clinical trials currently available.

Information regarding clinical trials for EGPA in other countries can be found at ClinicalTrials.gov; there may not be any clinical trials currently available.

For research and clinical trial updates, please visit ausEE Inc. – Clinical Trials.

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Rare Disease Organisation(s)

ausEE Inc. RVA Partner Australian Organisation
Website: https://ausee.org
Phone: 1300 923 043
Email:  [email protected]
Contact form: https://ausee.org/contactus/

ausEE Inc. is Australia’s peak national support and patient advocacy organisation representing Australians living with an eosinophilic disease, including EGPA and eosinophilic gastrointestinal diseases (EGIDs). ausEE’s mission is to improve the lives of those affected by eosinophilic diseases through providing support, evidence-based information, resources, advocacy and by campaigning to raise awareness and funds for research in Australia.

Australia and New Zealand Vasculitis Society (ANZVASC)RVA Partner Australian Organisation
Website: https://www.anzvasculitis.org/
Contact form: https://www.anzvasculitis.org/contact-us/

The Australian and New Zealand Vasculitis Society (ANZVASC) is a collaborative organisation of physicians, scientists, and communities that focuses on research, education, and clinical practices in vasculitis.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance.

Support Services/Resources

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

Mental Health

ausEE Inc. offers support programs, including for peer support.

Please visit the ‘Mental Health’ sections listed on the National and State Services pages.

Other

Further information on eosinophilic granulomatosis with polyangiitis (EGPA), and other vasculitis conditions, can be found at:

References
  1. Jennette JC, Falk RJ, Bacon PA,et al. 2012. Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. https://doi.org/10.1002/art.37715
  2. Chakraborty RK, Aeddula NR. Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome). 2024. Updated 23 March 2023. In: StatPearls [internet]. Treasure Island (FL): StatPearls Publishing; 2024 January. Accessed 15 August 2024. https://www.ncbi.nlm.nih.gov/books/NBK537099/
  3. National Organization for Rare Diseases. Eosinophilic Granulomatosis with Polyangiitis. Published 1989-2024. Updated 20 June 2024. Accessed 19 August 2024. https://rarediseases.org/rare-diseases/churg-strauss-syndrome/.
  4. Eosinophilic granulomatosis with polyangiitis. Updated October 2020. Accessed 15 August 2024. https://www.orpha.net/en/disease/detail/183.
  5. Australia and New Zealand Vasculitis Society (ANZVASC). Vasculitis types. Accessed 5 August 2024. https://www.anzvasculitis.org/vasculitis-types/
  6. Emmi G, Bettiol A, Gelain E, et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nature Reviews Rheumatology. 2023;19(6):378-93. https://doi.org/10.1038/s41584-023-00958-w
  7. American Lung Association. Learn About Eosinophilic Granulomatosis with Polyangiitis (EGPA). Updated 7 June 2024. Accessed 16 August 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/about-EGPA.
  8. American Partner for Eosininophilic Disorders, Vasculiits Foundation. Eosinophilic Granulomatosis with Polyangiitis: A Patient’s Guide to Resources and Hope. July 2024. 23p. https://www.vasculitisfoundation.org/wp-content/uploads/2024/07/egpa-patient-guide_FinalJuly24.pdf
  9. Vasculitis Foundation. About Eosinophilic Granulomatosis with Polyangiitis. Updated 5 February 2024. Accessed 13 August 2024. https://www.vasculitisfoundation.org/education/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis/
  10. American Lung Association. Symptoms and Diagnosis of EGPA. Updated 7 June 2024. Acccessed 19 August 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/symptoms-diagnosis.
  11. American Lung Association. Treating and Managing EGPA: American Lung Association. Updated 7 June 2024. Acccessed 19 August 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/treating-and-managing.
  12. Victoria State Government Department of Asthma and Smoking. Better Health Channel; 2024. Updated 22 September 2024. Accessed 2 September 2024. https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/asthma-and-smoking.
  13. Praveen Akuthota. What is Eosinophilic Granulomatosis with Polyangiitis? American Partner for Eosininophilic Disorders. Updated March 2020. Accessed 19 August 2024. https://apfed.org/about-ead/eosinophilic-granulomatosis-with-polyangiitis/.
  14. Genetic and Rare Disease Information Centre. Eosinophilic granulomatosis with polyangiitis. Updated July 2024. Accessed 13 August 2024. https://rarediseases.info.nih.gov/diseases/6111/index/diagnosis
Page Last Updated

09/09/2024 17:15