Clinical analysis of 25 cases of allergic granulomatous vasculitis

Abstract

To improve the understanding of allergic granulomatous vasculitis, also called Churg-Strauss syndrome (CSS). The clinical data, including clinical manifestations, laboratory findings, and pathology, of 25 patients with CSS were retrospectively investigated. There were 15 males and 10 females, aging 18 to 72 years (mean 45 ± 16). The clinical manifestations of CSS featured involvement of multiple systems or organs including respiratory system, nervous system, skin, digestive system, heart, and kidney. Eighty percent (20/25) of CSS patients had asthma as their first symptom, whereas 96.0% (24/25) had asthma in the whole course of CSS. The involvement of nasosinusitis of CSS was multi-group and the maxillary sinuses were the most frequently involved, which accounted for 61.1% (11/18). The peripheral nerves were involved in 72.7% (16/22) of patients and the most common manifestation was cerebrovascular event. Skin disease occurred in 60.0% (15/25), whereas heart involvement in 48.0% (12/25), renal involvement in 48.0% (12/25) and digestive system involvement in 32.0% (8/25) of the patients. The positive rate of IgE was 90.0% (18/20), and that of ANCA was 32.0% (8/25), with P-ANCA as the predominant. The main presentation of chest CT scan was patchy infiltration, mostly bilateral, which amounted for 71.4% (15/21). The change in electromyography was mononeuritis multiplex, of which the peroneal nerves and popliteal nerves were the most frequently involved. Electrocardiography of the CSS patients was always normal whereas the echocardiograms showed abnormal finds in 70.0% (14/20) of the patients. The pathological manifestations were necrotizing vasculitis, eosinophilic tissue infiltration, and extravascular granulomas. The clinical manifestations of CSS are diverse and complex, with a lack of pathognomonic symptoms. Clinical manifestations, auxiliary examinations and pathology are often required to make a diagnosis. Early diagnosis and treatment will be helpful for a better prognosis.