Approaches to the diagnosis of the Churg-Strauss syndrome

Abstract

Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a rare congenital autoimmune disease characterized by pathologies in the development of various organs. It develops either as a result of a genetic mutation (mutation of the CHD7 gene), or under the influence of external factors. The disease was first described in 1951 by Jacob Churg and Lotte Strauss as «allergic granulomatosis ». As a separate disease, the syndrome was isolated in 1990. The frequency of the disease ranges from 2 to 8 cases per 1 million people. There are no gender differences in the incidence of men and women. The disease begins to appear around the age of 38–49 years. The development of the syndrome goes through three stages. At the initial stage, allergic manifestations develop: rhinitis, sinusitis, hay fever, bronchial asthma, and drug intolerance. At the second stage, a hypereosinophilic syndrome, clinically manifested in Loeffler's syndrome, eosinophilic pneumonia, and eosinophilic gastroenteritis, develops. At the third stage, signs of systemic vasculitis appear.