Disease Information for Churg-Strauss syndrome

Widespread necrotizing angiitis with granulomas; Pulmonary involvement is usual; Asthma or other respiratory infection may precede evidence of vasculitis; Eosinophilia and lung involvement differentiate this disease from polyarteritis nodosa--------------------------------------------------Churg Strauss Syndrome; Allergic Angiitis and Granulomatosis; Allergic Granulomatosis; Allergic Granulomatosis and Angiitis; Churg-Strauss Vasculitis

Eosinophilic Granulomatous Vasculitis; Churg-Strauss Syndrome is a rare disorder that may affect multiple organ systems, particularly the lungs; The disorder is characterized by the formation and accumulation of an unusually large number of antibodies, abnormal clustering of certain white blood cells (eosinophilia), inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions (granulomatosis);Many individuals with Churg-Strauss Syndrome have a history of allergy; In addition, asthma and other associated lung (pulmonary) abnormalities ( pulmonary infiltrates) often precede the development of the generalized (systemic) symptoms and findings seen in Churg-Strauss Syndrome by one or more years; Asthma, a chronic respiratory disorder, is characterized by inflammation and narrowing of the lungs’ airways, causing difficulties breathing (dyspnea), coughing, the production of a high-pitched whistling sound while breathing (wheezing), and/or other symptoms and findings; Nonspecific findings associated with Churg-Strauss Syndrome typically include flu-like symptoms, such as fever, a general feeling of weakness and fatigue (malaise), loss of appetite (anorexia), weight loss, and muscle pain (myalgia); Additional symptoms and findings may be variable, depending upon the specific organ systems involved; Without appropriate treatment, serious organ damage and potentially life-threatening complications may result; Although the exact cause of Churg-Strauss Syndrome is unknown, many researchers indicate that abnormal immunologic and autoimmune factors play an important role[NORD 2005]-----------------------------------------------------

The American College of Rheumatology (ACR) has proposed 6 criteria for diagnosis of CSS. The presence of 4 or more criteria yields a sensitivity of 85% and a specificity of 99.7%. These criteria are (1) asthma (wheezing, expiratory rhonchi), (2) eosinophilia of more than 10% in peripheral blood, (3) paranasal sinusitis, (4) pulmonary infiltrates (may be transient), (5) histological proof of vasculitis with extravascular eosinophils, and (6) mononeuritis multiplex or polyneuropathy.

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