Secondary AA-amyloidosis in pediatric and adult patients: classification, etiology, pathogenesis, diagnosis, treatment

Abstract

The literature review summarizes information about the current nomenclature and classification of amyloidosis, the features of the etiology, pathogenesis, course, diagnosis and treatment of systemic secondary AA-amyloidosis in children and adult patients. Among the systemic forms of amyloidosis, secondary AA-amyloidosis accounts for 40–60 % of cases. The literature data on the features of the development of secondary AA-amyloidosis in familial Mediterranean fever (periodic illness) due to homozygous or heterozygous mutation of the MEFV gene in children are presented.