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CHURG-STRAUSS SYNDROME

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

Pathology

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 

Clinical Features

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 

Asthma 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

Purpura or nodules occur in approximately two thirds of the patients and reflect the preferential

Peripheral neuropathy, usually mononeuritis multiplex, is found in 64% to 75% of the patients. Its aspect

Polyarthralgias and arthritis frequently occur during the vasculitic phase of the disease. Any joint may be

Cardiac involvement is common in CSS and represents a major cause of mortality. It may consist of

GI 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 involvement is present in 16% to 49% of the patients. The characteristic glomerular lesion of 

Laboratory Tests

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

TREATMENT

Because Churg-Strauss syndrome responds well to In CSS, however, it is often impossible to

OUTCOME

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