PURPOSE: The purpose is to demonstrate that the diagnosis of Churg Strauss Syndrome can be made in pediatric patients without the presence of peripheral eosinophilia or necrotizing vasculitis.
METHODS: A 9 year old male caucasian child has been treated with singulair for 5 years because of eczema. Two years prior to the onset of this illnes, he developed mild persistent asthma and was treated by adding Fluticasone/Serevent. He presented with persistent cough. Chest X-rays showed progressive, migrating pulmonary nodules. Work up included Chest X-Rays, CT Scan of the chest, hematologic, Immunologic work up and biopsy of the lung. The biopsy showed several large non-caseating granulomas with ischemic necrosis. No hyalinization or calcification was noted. The background of the older granulomas was completely eosinophilic. Small amounts of nuclear dust were present in the more active appearing lesions. There were prominent eosinophilic infiltrates within the granulomas. Furthermore, eosinophils were present in the adjacent tissues, airway walls, periairway tissues and lobular parenchyma. No vasculitis was identified. No parasites were present. ANCA testing was negative.Since extravascular eosinophilic infiltrates are prominent feature of Churg Strauss syndrome, a therapeutic trial with oral steroids was carried out. This resulted in complete resolution of the symptoms and pulmonary nodules.
RESULTS: Complete resolution of the pulmonary nodules in a patient with second stage of Churg Strauss Syndrome prior to the appearance of peripheral eosinophilia and necrotizing vasculitis.
CONCLUSION: The diagnosis of Churg Strauss Syndrome can be made by lung biopsy findings including tissue eosinophilic infiltrates, non caseating granulomas and ischemic necrosis before the appearance of the pathognomonic findings of peripheral eosinophilia and vasculitis.
CLINICAL IMPLICATIONS: Churg Strauss Syndrome can occur in pediatrics. Early diagnosis before the presence of the pathognomonic features can result in complete resolution of the pulmonary granulomas and prevent permanent organ damage.Ref.1.Weller et al. The relationship os asthme therapy and Churg Strauss syndrome: NIH workshop summery report. J Allergy Clin Immunol. 2001;108:175-1832. Noth et al. Churg Strauss syndrome. Lancet. Feb. 15, 2003; 361(9357): 587-594.
DISCLOSURE: Louay Nassri, No Product/Research Disclosure Information; Grant monies (from industry related sources) Merck and Genetech; Consultant fee, speaker bureau, advisory committee, etc. Lecturer and Advisory board member for Merck, Schering, Sepracor, Astrazeneca and Tiva.