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

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

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

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

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

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

• Vasculitis
• Eosinophil
• Autoimmune disease

NIH genetic and rare disease info

• NIH info on Churg–Strauss syndrome

Churg–Strauss syndrome is a rare disease.

Churg–Strauss syndrome Resources
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