Churg-Strauss syndrome is a rare disorder that affects multiple organs especially the lungs. It is characterized by the abnormal clustering of certain white blood cells (hypereosinophilia) in the blood and tissues, inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions called granulomas (granulomatosis). Most of the affected individuals have a previous history of allergy. Asthma and other lung abnormalities often precede the development of the generalized (systemic) symptoms and findings seen in Churg-Strauss syndrome by as little as six months or as much as two decades.
Churg-Strauss disease is said to account for slightly more than 2% of all vasculitic illnesses. The various primary systemic vasculitides (Churg-Strauss disease, PAN, microscopic polyangiitis, Wegener granulomatosis) together are estimated to affect 15-25 individuals per million individuals in North America annually. Little information is available concerning international variation in the prevalence or incidence of Churg-Strauss disease.
Pathophysiology of Churg-Strauss syndrome include flu-like symptoms, such as fever, feeling of weakness and fatigue, loss of appetite, weight loss, and muscle pain. The nerves outside the central nervous system (peripheral nerves), kidneys, or gastrointestinal tract are often involved. The disorder is separated into three distinct phases – prodromal, eosinophilic and vasculitic.
The treatment of patients with Churg-Strauss syndrome is directed toward both immediately quieting the inflammation of the blood vessels (vasculitis) and suppressing the immune system. Treatment usually includes high doses of cortisone-related medication (such as prednisone or prednisolone) to calm the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan) and azathioprine. In many cases, the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and lifelong.
The ongoing research work related to Churg-Strauss syndrome include A Double-blind, Randomised, Placebo-controlled Study to Investigate the Efficacy and Safety of Mepolizumab in the Treatment of Churg-Strauss syndrome in Subjects Receiving Standard of Care Therapy, Cytokine production profile of CD4+ T cells from patients with active Churg-Strauss syndrome tends toward Th17, Longitudinal Protocol for Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) etc…