AA Amyloidosis in Familial Mediterranean Fever: a Comparative Study of Clinical and Genetic Features Between Algerian Probands and Their Relatives

AA amyloidosis is the most severe complication of familial Mediterranean fever (FMF). Why some patients develop amyloidosis while genotype-identical relatives remain unaffected is unknown. We assessed whether genetic counseling helps to identify at-risk relatives and whether inflammatory activity, rather than MEFV genotype, drives amyloidosis. We conducted a single-center comparative study (1998–2025) including 52 Algerian FMF probands with biopsy-proven AA amyloidosis and 30 first- or second-degree relatives with biallelic MEFV mutations who also had FMF but no amyloidosis. All underwent clinical, laboratory and MEFV genotyping (exons 2,3,5,10). Only individuals with two MEFV mutations (homozygous or compound heterozygous) were included. Multivariable logistic regression identified independent predictors of amyloidosis. The M694I/M694I genotype was equally frequent in both groups (71.2