INTRODUCTION: 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. DISCUSSION: 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. CONCLUSION: These cases may represent examples of a very puzzling relationship among disorders of innate and adaptive immune systems and inflammation.
INTRODUCTION: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. DISCUSSION: 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. CONCLUSION: These cases may represent examples of a very puzzling relationship among disorders of innate and adaptive immune systems and inflammation.
Authors: T Lequerré; O Vittecoq; P Saugier-Veber; A Goldenberg; P Patoz; T Frébourg; X Le Loët Journal: Rheumatology (Oxford) Date: 2006-12-18 Impact factor: 7.580
Authors: Silvia Mónica Meiorin; Graciela Espada; Christian Elias Costa; Alejandra Tartara; Elena De Matteo; Carine Wouters; Tammy M Martin; Carlos D Rose Journal: J Rheumatol Date: 2007-09 Impact factor: 4.666