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Churg-Strauss syndrome is a disorder characterized by pulmonary and systemic small vessel vasculitis, extravascular granulomas, and hypereosinophilia occurring in individuals with asthma and Churg-Strauss Syndrome, sindrome, Churg Strauss, Churg-Straus, Cherg-Strauss, Churg Straus
The two diagnostically essential lesions of CSS are angiitis and extravascular necrotizing granulomas with eosinophilic infiltrates. The vasculitis may be granulomatous or nongranulomatous, and characteristically involves both arteries and veins, as well as pulmonary and
There are three phases of the disease. A prodromal period, which may last for years (more than 30 years), consists of allergic manifestations of allergic rhinitis and nasal polyposis, frequently followed by asthma. The second phase of the disease is characterized by peripheral blood and tissue eosinophilia (with Loffler's syndrome), chronic eosinophilic pneumonia, or eosinophilic gastroenteritis. The eosinophilic infiltrative disease may remit and recur over years before the
AsthmaAsthma is a central feature of CSS and precedes the systemic manifestations in nearly all cases. In comparison with common asthma, it begins relatively late during life, at the age of 35. The severity and
Pulmonary Infiltrates
Pulmonary infiltrates may be present in nearly half the cases in the second phase of the disease, usually in association with asthma and hypereosinophilia mimicing chronic eosinophilic pneumonia. The
Cutaneous LesionsPurpura or nodules occur in approximately two thirds of the patients and reflect the preferential
NeuropathyPeripheral neuropathy, usually mononeuritis multiplex, is found in 64% to 75% of the patients. Its aspect
Musculoskeletal InvolvementPolyarthralgias and arthritis frequently occur during the vasculitic phase of the disease. Any joint may be
Cardiac InvolvementCardiac involvement is common in CSS and represents a major cause of mortality. It may consist of
Gastrointestinal InvolvementGI tract symptoms occur in 37% to 62% of the patients. They include abdominal pain, diarrhea, and GI bleeding. Two different mechanisms of involvement are possible. Mesenteric vasculitis is
Renal InvolvementRenal involvement is present in 16% to 49% of the patients. The characteristic glomerular lesion of
Eosinophilia is constant and often greater than 109 per L. The absence of eosinophilia may be explained by prior corticosteroid treatment for asthma. The association of eosinophilia greater than 109 /L with
CSS is strongly associated with ANCA, primarity myeloperoxidase, which was found in 67% of the
Because Churg-Strauss syndrome responds well to In CSS, however, it is often impossible to
Vasculitides of the PAN group are acute or subacute diseases, and it is possible to successfully treat patients and to definitively stop treatment. Nevertheless, relapses can occur. They are rare in
Sequelae can persist, but often cannot be predicted. Renal insufficiency is the consequence of vasculitis or glomerulonephritis; some patients may need chronic dialysis. Renal insufficiency can occur years after