Churg Strauss Syndrome: Clinical and Pathogenetic Approach to Therapy

Abstract

The Churg-Strauss syndrome (CSS), also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. CSS is classified as a vasculitis of the small and medium sized arteries, which commonly involve the lung, the skin and the peripheral nerves and it is associated to ANCA in 40-60 percent (Sinico & Bottero 2009). Among the three anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (CSS, Wegener's granulomatosis, and microscopic polyangiitis), CSS is least common. In the general population, the frequency of the disorder has been estimated at 2.4–6.8 per 1 000 000 patient-years, while the incidence of CSS in asthma drug users has been estimated to be 14-52 per million person-years (Watts RA et al. 2000). The mean age at diagnosis of CSS is 40 years, with no gender predominance. Diagnosis of Churg-Strauss syndrome can be difficult, because the syndrome may arise at first as a common association between asthma and allergic rhinitis. Because asthma itself might be associated with sinusitis, occasional pulmonary infiltrates (eg, mucus plugging, atelectasis, or intermittent infection), and corticosteroid dependency, a clear diagnosis of Churg-Strauss syndrome might not be made until extra respiratory system become involved, such as the abdominal viscera, heart, or nervous system.