Necrotizing sarcoid granulomatosis in a family of patients with sarcoidosis reinforces the association between both entities.
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.
Lazzarini
Luiz Claudio Oliveira
LC
Institute of Thoracic Diseases, Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. lazzarini@hucff.ufrj.br
de Fatima do Amparo Teixeira
Maria
M
Souza Rodrigues
Rosana
R
Marcos Nunes Valiante
Paulo
P
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
D000305:Adrenal Cortex Hormones