EGPA The Prologue.

So what is it?

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Just asking.

What is EGPA?

Well the question is simple, the answer can be polymorphic.

'It's a type of Vasculitis' or 'An autoimmune condition' or 'A Syndrome' aren't necessarily helpful unless you are already an expert in those areas.

'It's a disease attacking blood vessels, causing organ damage' is a lot more descriptive but not complete or very satisfying, particularly to the medical profession who like to be more precise about these things.

Who is asking?

It also depends on the reason for the question

A sufferer might describe it as aggressive asthma, extreme fatigue, terrible neuropathy or other things, depending on which symptoms predominate at the time.

A rheumatologist might have a different perspective to a neurologist and so on.

Where are the answers?

This section aims to provide some background, give reasons for today's definitions and answers to some of the fundamental questions.


The Cat: So, what is it?
Kryten: I've never seen it before, not many have, but I'm guessing it's a form of small vessel vasculitis.
Rimmer: Vasculitis?
Kryten: Yes, the classical clinical phenotype is leukocytoclastic with palpable purpura, but manifestations vary widely depending upon the organs involved. Histopathologic examination reveals angiocentric segmental inflammation, fibrinoid necrosis, and a neutrophilic infiltrate around the blood vessel walls with erythrocyte extravasation.
The Cat: So, what is it?
A parody on BBC Red dwarf season 4 'White hole'

OK, so what is it?

Defining moments.

When Churg and Strauss did their work in the 1950's, the disease was as yet undefined, mortalities being put down to Polyarteritis Nodosa (PAN) or maybe just 'unknown'.

Following their detailed studies they concluded a new syndrome comprising severe asthma, fever, hyper-eosinophilia and granulomatous lesions both in Vessel walls and connective tissue.[1]

They called it 'allergic granulomatosis'. This nomenclature arose primarily because they were investigating cases which presented with severe asthma, granulomatous extravascular lesions and inflammatory, granulomatous vascular changes.

Syndrome.

These physical presentations are simply a set of common characteristics and the meaning of the word 'Syndrome', A syndrome is often named after its discoverers, hence Churg-Strauss Syndrome (CSS) was eventually coined.
It will be referred to as EGPA from hereon for consistency, accepting the incorrect historic context.

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