An Association between MEFV Mutation and ANCA-Associated Vasculitis Involving the Central Nervous System and Lungs: Causality Under Scrutiny.

Unlabelled: Familial Mediterranean fever (FMF) is traditionally associated with biallelic mutations in the MEFV gene; however, heterozygous mutations may also contribute to disease phenotypes. We report the case of a 42-year-old woman with heterozygous p.Met694Ile MEFV mutation, presenting with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system and lungs.