Churg-Strauss's Syndrome; Review of literature

Abstract

Churg – Strauss Syndrome (CSS) is a rare idiopathic, multisystemic, autoimmune disease, characterized by diffuse eosinophilic infiltration and necrotizing vasculitis of small to medium sized vessels. Late onset asthma and/or allergic rhinitis are frequent clinical manifestations with fever and loss of weight as systemic symptoms at presentation. Mononeuritis multiplex, myalgia, skin involvement (purpura, skin nodules, urticarial rash, livedo reticularis), arthralgia, gastro intestinal tract (GIT) involvement, pericardial, myocardial, central nervous system (CNS) and renal involvements are other manifestations of CSS. Diagnostic hallmarks include Blood and tissue eosinophilia, histology revealing eosinophilic granulomas and/or necrotizing vasculitides in tissue biopsy. Although not a criterion of CSS diagnosis, presence of Antineutrophil Cytoplasmic Antibodies (ANCA), especially antimyeloperoxidase (ANCA MPO), is associated with CSS. This is present in more than 40% (40 - 66 - 73%) of CSS patients. Pathogenesis varies from ANCA activation of neutrophils and resultant systemic endothetial damage (in ANCA positive CSS), on one hand, and tissue infiltration by eosinophils with resultant tissue damage by toxic products of eosinophils (in ANCA negative CSS), on the other hand. There are also the varying B and T cell dysfunctions. Diffuse organ involvement, especially cardiovascular (CVS), GIT, rare involvement of CNS and renal system suggest poorer prognosis and possible fatal outcome. Patients with these poor prognostic outlooks, especially cardiac, receive aggressive treatment using corticosteroids combined with immunosuppresants (chiefly cyclophosphamide). Patients with refractory CSS have been successfully treated, adding TNF – alpha blockers to a combination of glucocorticoids and cyclophosphamide. Antithymocyte globulin or pooled intravenous globulin have been found effective alternative therapy in resistant cases and most especially in pregnant patients.

Keywords: Churg-Strauss syndrome, Eosinophilia, Antineutrophil Cytoplasmic Antibodies, Immunosuppressants