Eosinophilic granulomatosis with polyangiitis (EGPA, but previously known as Churg–Strauss syndrome (named after Doctors Jacob Churg and Lotte Strauss)) is an autoimmune vasculitis, leading to necrosis. It involves small and medium blood vessels mainly of the lungs, gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys. It's a rare disease that is non-inheritable and non-transmissible.
There are three stages to EGPA:
- The allergic phase
- The eosinophilic phase (raised eosinophils are found at this point)
- The vasculitic phase
These phases may occur sequentially or concomitantly.
It's known to cause severe asthma and treatment involves immunosuppressive agents such as steroids, cyclophosphamide, steroid-sparing agents (DMARDs such as azathioprine and methotrexate), as well as monoclonal antibodies (such as mepolizumab and benralizumab which decrease levels of eosinophils via different mechanisms).