title: Autoinflammatory diseases: mimics of autoimmunity or part of its spectrum? Case presentation.
abstract: BACKGROUND
  Autoinflammatory diseases are very rare diseases presenting within a wide clinical spectrum. Recognition of the main clinical features are challenging due to overlapping or mimicking with autoimmune diseases.
  
  
  CONCLUSIONS
  A case series is reviewed to illustrate typical and atypical features and the difficulties of these diagnoses in the low prevalence areas--a typical unrecognized case of familial Mediterranean fever (FMF) in a youngster, an atypical adult case with overlapping of FMF with Behçet disease, and an early presentation of FMF in infant presenting with inflammatory colitis, as well as the overlapping features within the cryopirin diseases spectrum in an 8-year-old boy who presented with systemic onset arthritis.
  
  
  CONCLUSIONS
  These cases may represent examples of a very puzzling relationship among disorders of innate and adaptive immune systems and inflammation.
authors: Rheumatology Ph.D, University of São Paulo Medical School, São Paulo, Brazil. mhbkiss@terra.com.br
authors: Maria Helena B
authors: MH
authors: Kiss
authors: Claudia Saad
authors: CS
authors: Magalhães
mesh_terms: D000328:Adult; D001168:Arthritis / Q000175:diagnosis / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D015551:Autoimmunity; D001528:Behcet Syndrome / Q000175:diagnosis / Q000276:immunology; D002352:Carrier Proteins / Q000235:genetics* / Q000276:immunology; D002648:Child; D003092:Colitis / Q000175:diagnosis / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000235:genetics* / Q000276:immunology; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007113:Immunity, Innate; D007223:Infant; D007249:Inflammation; D008297:Male; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D000071198:Pyrin
chemical_list: D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; C000604192:MEFV protein, human; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human; D000071198:Pyrin