title: Pulmonary Necrotizing Granulomas in a patient with familial mediterranean fever.
abstract: We herein report a case of familial Mediterranean fever (FMF) presenting with granulomatous lung lesions with neuronal apoptosis inhibitory protein (NAIP), MHC class II transcription activator (CIITA), incompatibility locus protein from Podospora anserina (HET-E), and telomerase-associated protein (TP1) (NACHT) leucine-rich-repeat 1-positive inflammatory cell infiltrates. FMF is an autoinflammatory disorder characterized by recurrent and self-limited attacks of pyrexia, arthritis and erysipelas-like skin lesions. Lung disorders associated with FMF are extremely rare. This is the first report of an immunologically-confirmed case of pulmonary manifestations of this disease.
authors.affiliation: a Department of Respiratory Medicine , Oita University Hospital , Oita , Japan.
authors.forename: Hisako
authors.initials: H
authors.lastname: Kushima
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishii
authors.forename: Koji
authors.initials: K
authors.lastname: Ishii
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Kadota
mesh_terms: D001706:Biopsy; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D005260:Female; D015769:Granuloma, Respiratory Tract / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D013601:T-Lymphocytes / Q000276:immunology; D014057:Tomography, X-Ray Computed; D043222:Ubiquitin Thiolesterase / Q000276:immunology* / Q000378:metabolism
chemical_list: C482860:UCHL1 protein, human; D043222:Ubiquitin Thiolesterase
keywords: Immunohistochemistry; Lung; familial Mediterranean fever