POS1372 EVALUATION OF E148Q AND CONCOMITANT AA AMYLOIDOSIS SECONDARY TO FAMILIAL MEDITERRANEAN FEVER AFTER ADJUSTED CLINICAL-DEMOGRAPHIC CHARACTERISTICS

Background: Amyloid A (AA) amyloidosis, previously known as secondary or reactive is a long-recognized severe complication of some chronic inflammatory diseases. The pathogenesis and risk factors for amyloidosis in Familial Mediterranean Fever (FMF) remain partially understood (1). development AA dependent on ethnicity country residence (2). In the pre-colchicine era, renal AA-amyloidosis was largely reported patients Turkish (67%) Sephardic Jewish ancestry (26.5%) (2,3). Currently it’s well that MEFV M694V variant associated with high however, mutations exon 2, specifically E148Q remained controversial. Objectives: To evaluate mutation concomitant Amyloidosis to FMF after adjusted clinical-demographic characteristics. Methods: Patients were recruited from unit at Epigenetic Health Center outpatient clinic Turkey between September 2003 February 2020. who had biopsy confirmed related included study. Tel-Hashomer criteria applied diagnosis FMF. clinical characteristics medication history recorded by physician time registry entry. All detailed baseline assessment features, functions, genetic testing, histopathological treatment received. We performed multiple comparisons according age diagnosis, demographic disease phenotype, allele frequency, type mortality. Statistical analysis Package Social Science (SPSS) Windows, version 15.0 (SPSS Inc, Chicago, IL). Results: Our consists 195 amyloidosis. Complete information 169 (lost follow up, n=26) included. median 36 (19-49) years; male/female ratio 1.6 (104/65). follow-up duration years (4-17 years). There 101 diagnosed <18 68 ≥18 age. participants developed before 32 years. Family documented 56 (33.1%) family present 41 (24.3%). three most common symptoms fever (84,6%), abdominal pain (71.6%) arthritis (66.9%). During follow-up, 5 started dialysis 9 kidney transplantation. frequency across (60.6%), (21.4%) M680I (10.3%). frequent M694V/M694V (63.3%), M694V/E148Q (20.8%) E148Q/E148Q (15.8%). up period, 15 (10 male, female) died. those died, 14 M694V/M694Vand one demonstrated E148Q/E148Q. Conclusion: have an increased This study association FMF-associated which has been many studies. Close further evaluation warranted if residing endemic areas. possible relationship need be other cohorts. References: [1]Erer B, Demirkaya E, Ozen S, Kallinich T. What best acute phase reactant familial its role prediction complications? systematic review. Rheumatology international. 2016;36(4):483-7. [2]Touitou I, Sarkisian T, Medlej-Hashim M, Tunca Livneh A, Cattan D, et al. Country primary factor fever. Arthritis rheumatism. 2007;56(5):1706-12. [3]Pras Bronshpigel N, Zemer Gafni J. Variable incidence among different ethnic groups. Johns Hopkins Med 1982;150(1):22-6. Disclosure Interests: None declared