A case of Churg-Strauss syndrome presenting with acute respiratory failure due to diffuse pulmonary hemorrhage / 천식및알레르기

ABSTRACT

Churg-Strauss syndrome is a rare subset of the group of systemic necrotizing vasculitis. Acute respiratory failure is an extremely rare presentation of Churg-Strauss syndrome. The authors report a case of Churg-Strauss syndrome presenting with acute respiratory failure due to diffuse pulmonary hemorrhage. A 28-year-old male suffering from not-well responding bronchial asthma developed erythematous maculopapulous skin lesion and progressive infiltrative pulmonary consolidation on chest X-ray but initially there was no eosinophilia in the peripheral blood. One week later, acute respiratory failure developed with peripheral eosinophilia. Chest X-ray showed bilateral multiple consolidations and patchy densities. The patient was supported on mechanical ventilator and we performed skin biopsy and bronchoscopic examination immediately. The lung biopsy specimens demonstrated capillaritis and prominent interstitial and alveolar eosinophil infiltrates. Grossly bloody bronchoalveolar lavage fluid showed predominant increase in eosinophilic increase and many hemosiderin laden macrophages. Therefore the patients was diagnosed with Churg-Strauss syndrome presenting with pulmonary hemorrhage. Treatment with intravenous methylprednisolone produced symptomatic improvement and normalized peripheral eosinophilia. This case emphasizes that Churg-Strauss syndrome should be considered in the differential diagnosis of acute respiratory failure, and early intervention or diagnostic approach is needed.