title: Necrotizing sarcoid granulomatosis of the lung in a 12-year-old boy with an atypical clinical course.
abstract: Necrotizing sarcoid granulomatosis (NSG) is a disorder of unknown etiology, rarely described in childhood, belonging to the heterogeneous group of the pulmonary angiitis and granulomatosis. One of the characteristics of NSG is to have typically a benign clinical course with minimal treatment with systemic steroids or even with no therapy at all. Here, we report the case of a boy with a lung consolidation, with morphological and histological features consistent with a diagnosis of NSG. Good clinical and roentgenographic response to high dose prednisone treatment was followed three times by relapses, when steroid treatment was tapered. New lesions were detected in different areas of the lung and not in initially affected area, never previously described in NSG and only rarely in other pulmonary angiitides.
authors.affiliation: Pulmonary and Allergy Disease Paediatric Unit, G Gaslini Institute, Genoa, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Panigada
authors.forename: Nicola
authors.initials: N
authors.lastname: Ullmann
authors.forename: Oliviero
authors.initials: O
authors.lastname: Sacco
authors.forename: Claudio
authors.initials: C
authors.lastname: Gambini
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.forename: Giovanni A
authors.initials: GA
authors.lastname: Rossi
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D006099:Granuloma / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008171:Lung Diseases / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D009336:Necrosis / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D017565:Sarcoidosis, Pulmonary / Q000188:drug therapy / Q000473:pathology*; D014657:Vasculitis / Q000188:drug therapy / Q000473:pathology*
chemical_list: D000893:Anti-Inflammatory Agents; D011241:Prednisone