Churg–Strauss syndrome
Churg–Strauss syndrome (CSS), also known as Eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune disease characterized by inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).
Etiology[edit | edit source]
The exact cause of Churg-Strauss syndrome is unknown. However, it is believed to be an autoimmune disorder, a condition in which the body's immune system mistakenly attacks its own cells and tissues. Some researchers believe that the syndrome may be triggered by an allergic reaction.
Symptoms[edit | edit source]
Symptoms of Churg-Strauss syndrome can vary greatly from person to person. They may include asthma, sinusitis, skin rashes, joint pain, abdominal pain, and high numbers of a specific type of white blood cell called an eosinophil.
Diagnosis[edit | edit source]
Diagnosis of Churg-Strauss syndrome is based on a combination of symptoms, physical examination, and medical history. Laboratory tests may show an increased number of eosinophils. Other tests may include a chest X-ray, a CT scan of the lungs, or a biopsy of affected tissue.
Treatment[edit | edit source]
Treatment for Churg-Strauss syndrome typically involves medications to reduce inflammation and suppress the immune system. These may include corticosteroids and other immunosuppressive drugs. In severe cases, chemotherapy may be used.
Prognosis[edit | edit source]
The prognosis for individuals with Churg-Strauss syndrome varies. Some people may experience long periods of remission, while others may have recurring episodes of the disease. Early diagnosis and treatment can improve the prognosis.
See also[edit | edit source]
References[edit | edit source]
NIH genetic and rare disease info[edit source]
Churg–Strauss syndrome is a rare disease.
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Contributors: Prab R. Tumpati, MD