eosinophilic granulomatosis with polyangitis or egpa previously known as chur Strauss syndrome is a rare immune disorder that causes systemic organ damage from eosinophilic involvement and inflammation of small blood vessels called vasculitis it is frequently associated with allergic manifestations like asthma ritis and sinusitis egpa is characterized by the presence of increased amounts of eosinophils in the blood eosinophils are a type of white blood cell that play a role in the body's response to allergens and parasitic infections it's unclear how egpa develops but it's thought that some kind of trigger such as an infection medication or environmental allergen sets off an exaggerated eosinophilic immune response that causes damage to tissues and organs some people may be more susceptible to developing egpa when not treated egpa leads to the development of granulomas which are collections of immune cells that cluster together to wall off an area of inflammation signs and symptoms of egpa can vary greatly from one individual to another but there are typically three phases prodromal eosinophilic and vasculitic although they don't always occur in that order the prodromal phase is characterized by adult onset asthma and ritis that are often attributed to allergies unlike asthma egpa often has Progressive lung involvement commonly with infiltrates in addition chronic inflammation of the nasal lining causes growth of tissue within the nasal cavity called polyps these polyps contain large amounts of eosinophils and can block the sinus tracts preventing mucus from draining out of the sinuses and causing congestion or inability to smell the eosinophilic phase results in infiltration of eosinophils into organs and can cause characteristic lesions called eosinophilic granulomas when eosinophils infiltrate the lungs pulmonary infiltrates can form that are seen on radiologic Imaging eosinophils can also affect the heart lining and muscle and cause heart damage which in severe cases can result in heart failure in some patients clots can form in the blood vessels or in the heart there may also be gastrointestinal or GI symptoms including abdominal pain diarrhea as a result of eosinophils in the lining of the stomach or small bowel and more rarely blood in the stool due to inflammation of the GI tract other organs can also be affected such as the kidneys especially in patients with an autoimmune type of egpa who have an antibody called Anka that is present in the blood the most severe presentation of egpa is the vasculitic phase when vasculitis occurs during this phase of the disease a necrotizing vasculitis that affects smaller vessels supplying peripheral nerves occurs clinically this manifests as nerve pain and loss of sensation and or muscle strength which in some patients can lead to loss of muscle control of the foot or wrist in addition the inflamed blood vessels become more fragile potentially rupturing and bleeding into the surrounding tissue this can cause a peric rash which is seen as purple or red spots on the skin caused by Blood pooling underneath finally while less common individuals with egpa may present with a rapid decline in kidney function or BL bloody urine due to renal disease diagnosis of egpa can be challenging due to the numerous clinical presentations and lack of diagnostic markers in general diagnosis requires a high index of Suspicion and is often considered in individuals who present with adult onset asthma rhinosinusitis and eosinophilia together additional examinations may include chest Imaging such as a CT scan which may show transient migratory lung infiltrates which are opaque densities that appear in different locations a bronchoscopy or a tissue biopsy which may reveal large quantities of eosinophils in or around blood vessels around 30 to 50% of affected individuals also have Anka Auto antibodies in their blood which may also Aid the diagnosis most individuals with egpa are first treated with corticosteroids such as prazone to suppress the activity of the immune system and help reduce inflammation if individuals do not respond to corticosteroid therapy alone or if they have advanced disease they may require additional immunosuppressive medications like cyclophosphamide or rmab to achieve remission other immune suppressive medications such as Methotrexate can be used to maintain control of the disease biologic treatments that block a protein called il5 like mepolizumab or the il5 receptor like benralizumab have been shown to help in non- severe egpa to maintain remission in place of classic immunosuppressive medications well most individuals improve with current treatment strategies some patients require long-term corticosteroid therapy in addition to other medications because of persistent asthma symptoms as a quick recap egpa is a rare immune disorder characterized by systemic small vessel vasculitis often preceded by allergic manifestations like asthma rinitis and sinusitis diagnosis is based on clinical manifestations and evidence of a high eosinophil count in the blood or a tissue biopsy treatment is focused on minimizing inflammation with corticosteroids immunosuppressive medications and newer biologic Therapies