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

STUDY OBJECTIVES: To describe the radiologic and clinical findings of Churg-Strauss syndrome (CSS) and its thoracic manifestations. DESIGN: We used retrospective analysis to review and characterize the radiographic, thin-section CT, and clinical findings of CSS. PATIENTS: 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. RESULTS: 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). CONCLUSIONS: 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.