title: Necrotizing sarcoid granulomatosis in a family of patients with sarcoidosis reinforces the association between both entities.
abstract: Necrotizing sarcoid granulomatosis (NSG) is a rare entity mainly characterized by a prominent granulomatous vasculitis affecting middle-aged or old individuals and with a favorable prognosis. Although many believe it is a variant of sarcoidosis, the proper classification is still a matter of debate as some of its features are found in sarcoidosis but also in Churg-Strauss syndrome, Wegener's disease and hypersensitivity pneumonitis. In this paper, we described for the first time a case of NSG in a family with several cases of sarcoidosis, reinforcing the relationship between NSG and sarcoidosis. Additional interesting findings were the young age of the patient (15 years old), the symptoms limited to the respiratory tract (uncommon when NSG affects youngsters) and the increase in serologic markers of autoimmune disease. Though complete criteria for autoimmune disease were not present, systemic lupus erythematosus and Sjogren's syndrome are possible candidates. As sarcoidosis is described to be associated with several autoimmune diseases, this finding is an additional suggestion of the relationship between both entities.
authors: Institute of Thoracic Diseases, Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. lazzarini@hucff.ufrj.br
authors: Luiz Claudio Oliveira
authors: LC
authors: Lazzarini
authors: Maria
authors: M
authors: de Fatima do Amparo Teixeira
authors: Rosana
authors: R
authors: Souza Rodrigues
authors: Paulo
authors: P
authors: Marcos Nunes Valiante
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D005260:Female; D015769:Granuloma, Respiratory Tract / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology*; D008875:Middle Aged; D009336:Necrosis; D010375:Pedigree; D017565:Sarcoidosis, Pulmonary / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones