What is the definition of Eosinophilic Granulomatosis with Polyangiitis?

Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) is a condition characterized by asthma, high levels of eosinophils (a type of white blood cell that helps fight infection), and inflammation of small to medium sized blood vessels (vasculitis). The inflamed vessels can affect various organ systems including the lungs, gastrointestinal tract, skin, heart and nervous system. The exact cause of eosinophilic granulomatosis with polyangiitis is unknown, but it is thought to be an autoimmune disorder. Treatment may involve the use of glucocorticoids (steroids) and/or other immunosuppressive therapies. As of December 2017, mepolizumab (Nucala) became the first therapy approved specifically to treat eosinophilic granulomatosis with polyangiitis. When added to traditional steroid or immunosuppressive therapy, mepolizumab has been found to increase remission rate and time in remission for 50% of people with eosinophilic granulomatosis with polyangiitis.

What are the alternative names for Eosinophilic Granulomatosis with Polyangiitis?

  • CSS
  • Allergic granulomatous and angiitis
  • Allergic angiitis and granulomatosis
  • Allergic granulomatosis
  • Churg-Strauss vasculitis
  • Granulomatous allergic angiitis
  • Churg-Strauss syndrome
  • EGPA

What are the symptoms for Eosinophilic Granulomatosis with Polyangiitis?

The specific signs and symptoms of eosinophilic granulomatosis with polyangiitis vary from person to person depending on the organ systems involved. The severity, duration and age of onset also vary. Eosinophilic granulomatosis with polyangiitis is considered to have three distinct phases - prodromal (allergic), eosinophilic and vasculitic - which don't always occur sequentially. Some people do not develop all three phases.
  • The prodromal (or allergic) phase is characterized by various allergic reactions. Affected people may develop asthma (including a cough, wheezing, and shortness of breath); hay fever (allergic rhinitis); and/or repeated episodes of sinusitis. This phase can last from months to many years. Most people develop asthma-like symptoms before any other symptoms.
  • The eosinophilic phase is characterized by accumulation of eosinophils (a specific type of white blood cell) in various tissues of the body - especially the lungs, gastrointestinal tract and skin.
  • The vasculitic phase is characterized by widespread inflammation of various blood vessels (vasculitis). Chronic vasculitis can cause narrowing of blood vessels, which can block or slow blood flow to organs. Inflamed blood vessels can also become thin and fragile (potentially rupturing) or develop a bulge (aneurysm).
Depending on the organ affected in the disease, other symptoms may include:
  • Nonspecific symptoms: Many people may have nonspecific symptoms such as fatigue, fever, weight loss, night sweats, abdominal pain, and/or joint and muscle pain. 
  • Respiratory problems: Asthma, often with onset during adulthood, occurs in most patients and is often severe. Other symptoms are sinusitis, difficult breathing, cough and coughing up of blood (hemoptysis), and transient patchy lung lesions.
  • Neurological symptoms: Neurological symptoms such as pain, tingling or numbness and are common and depend on the specific nerves involved. 
  • Skin symptoms: About half of affected people may develop skin abnormalities such as purplish skin lesions, a rash with hives, and/or small bumps (especially on the elbows) due to accumulation of eosinophils in skin tissue. 
  • Gastrointestinal symptoms: About one third of affected people present with GI symptoms (abdominal pain, diarrhea, bleeding, acalculous cholecystitis) due to eosinophilic gastroenteritis or mesenteric ischemia due to vasculitis
  • Heart problems: Heart problems may include inflammation of heart tissues (myocarditis and endomyocardial fibrosis) and in severe cases, heart failure. 
  • Muscular and bone problems: Joint pain (arthralgias), muscular pain (myalgias), or even arthritis can occur, usually during the vasculitic phase. Kidney problems: The kidneys are affected less often but can also become involved, eventually causing glomerulonephritis.

What is the outlook (prognosis) for Eosinophilic Granulomatosis with Polyangiitis?

The prognosis may still depend on the severity of the illness in each person and the specific organ systems that are affected. In general, the prognosis has improved significantly since the use of corticoids and selected use of immunosuppressant agents for people with more severe disease.  Although eosinophilic granulomatosis with polyangiitis can be progressive and serious, many affected people do very well.   If treatment is started early and the disease is carefully monitored, organ damage may be reduced. Even people with very severe cases can get well (remission) when treated promptly and followed closely. However, it is possible for eosinophilic granulomatosis with polyangiitis to recur. People with asthma or nasal allergies often have worsening of these symptoms, independent of vasculitis. Relapses involving vasculitis occur in about 30% to 50% of affected people. The chance to have a severe relapse can be minimized by prompt reporting of any new symptoms. Relapses are treated the same way as when the disease started for the first time, and it is possible to achieve remission again after a relapse. Most deaths in affected people are due to complications from the vasculitic phase of eosinophilic granulomatosis with polyangiitis and are most commonly due to heart failure and/or heart attack; kidney failure; cerebral bleeding; gastrointestinal bleeding; and acute, or severe asthma (status asthmaticus).
  • Condition: Patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA)
  • Journal: Arthritis research & therapy
  • Treatment Used: Mepolizumab in Combination with Corticosteroids
  • Number of Patients: 16
  • Published —
This study assessed the effectiveness and safety of mepolizumab (MPZ) for patients with relapsing/refractory eosinophilic granulomatosis with polyangiitis (vascular inflammation; EGPA).