Have I got EGPA?

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Getting medical confirmation.

The problem.

EGPA is a small blood vessel vasculitis, which also affects some medium sized vessels and nearly always starts with asthma, often accompanied by allergic rhinitis. This itself does not confirm a positive diagnosis! After all, approximately 10% of the UK population is affected by asthma.[1]

The three phases.

It was found as long ago as 1984 that EGPA often progresses in phases [2]. The first (prodromal phase) is indeed characterised by asthma and rhinitis, the second (eosinophilic phase) is characterised by high eosinophil levels in blood and organ tissue such as the lungs. Detection would require blood tests and more significantly a biopsy, something unlikely to be conducted without good reason. High eosinophils in the blood are found in other diseases too!

The first stage can last for years, the second normally much shorter and can overlap. Finally the vasculitic phase can happen very quickly, even overnight, often resulting in hospitalisation.

The six criteria.

The American College of Rheumatology in 1990 identified six criteria for the classification of EGPA, where the presence of four or more of these confirm a strong probability of EGPA.[4].

Original work on EGPA.

Dr. Jacob Churg spent more than 50 years at the Icahn School of medicine Mount Sinai, New York,[24] where he completed more than 300 publications. He collaborated with Dr. Lotte Strauss in studying allergic granulomatosis, later to be known as Churg-Strauss syndrome and now EGPA. He also made major discoveries in the field of renal pathology.
Aside from her work with Dr Churg, Dr Strauss was one of the pioneers in pediatric and perinatal pathology and one of the founders of the Society for Pediatric Pathology. She published around 100 scientific articles.
The ground-breaking paper was published in 1951.[3]

Getting organised.

Establish the basics.

If you have medical confirmation or think you might have EGPA, it is important that you quickly establish good rapport with your medical team.

Read as much as you can about your condition, symptoms and medication options.

Seek out the best.

If you are lucky enough to have access to best practice medical facilities, make the most use of it, if not then be prepared to travel or work much harder on your options.

Use others exerience on social media sites to find out where centres of excellence are. Weigh this up against what you read and what your local medical team suggests.

Understand the urgency.

Be clear that at one extreme,early diagnosis and treatment can lead to a normal, active life, at the other it can be fatal.

It is therefore important to be diagnosed as early as possible, a task admittedly made more difficult by the absence of a simple test.

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