Thoracic manifestation of Churg-Strauss syndrome : Radiologic and clinical findings

To describe the radiologic and clinical findings of Churg-Strauss syndrome (CSS) and its thoracic manifestations. We used retrospective analysis to review and characterize the radiographic, thin-section CT, and clinical findings of CSS. The study involved nine patients with CSS. The patients included four men and five women, whose ages ranged from 18 to 60 years (median, 35 years). Thin-section CT scans and chest radiographs were retrospectively analyzed by three radiologists in consensus. Clinical data were obtained by chart review. Histologic samples were available in eight patients. All patients had a history of asthma averaging 28 months (range, 4 to 72 months) prior to the initial symptom of vasculitis and marked peripheral blood eosinophilia (mean peak count, 8,726/microL; range, 3,000 to 32,000/microL; mean differential count, 41%; range, 19 to 67%). All patients had systemic vasculitis involving the lung and two to four extrapulmonary organs, most commonly the nervous system (n = 8) and skin (n = 7). Chest radiographs showed bilateral nonsegmental consolidation (n = 5), reticulonodular opacities (n = 3), bronchial wall thickening (n = 3), and multiple nodules (n = 1). The most common thin-section CT findings included bilateral ground-glass opacity (n = 9); airspace consolidation (n = 5), predominantly subpleural and surrounded by the ground-glass opacity; centrilobular nodules mostly within the ground-glass opacity (n = 8); bronchial wall thickening (n = 7); and increased vessel caliber (n = 5). Other findings were hyperinflation (n = 4), larger nodules (n = 4), interlobular septal thickening (n = 2), hilar or mediastinal lymph node enlargement (n = 4), pleural effusion (n = 2), and pericardial effusion (n = 2). In CSS, thoracic organs are invariably involved with additional diverse manifestations. The possibility of CSS should be raised in patients with a history of asthma and hypereosinophilia who present with thin-section CT findings of bilateral subpleural consolidation with lobular distribution, centrilobular nodules (especially within the ground-glass opacity) or multiple nodules, especially in association with bronchial wall thickening.