title: Endothelial function in patients with familial Mediterranean fever-related amyloidosis and association with cardiovascular events.
abstract: OBJECTIVE
  Secondary amyloidosis is the most important complication of FMF and endothelial function is more severely impaired. Elevated asymmetric dimethyl arginine (ADMA) may mediate the excess cardiovascular disease (CVD) risk of this group. We aimed to compare endothelial function characteristics, including ADMA, in patients with FMF-related amyloidosis and primary glomerulopathies and to define risk factors for a CVD event.
  
  
  METHODS
  We undertook a cross-sectional study with prospective follow-up including consecutive patients with FMF-related amyloidosis (n = 98) or other non-diabetic glomerulopathies (n = 102). All patients had nephrotic-range proteinuria and normal glomerular filtration rate. Flow-mediated dilatation (FMD) was assessed and ADMA levels, CRP and pentraxin 3 (PTX3) were determined. Patients were followed for cardiovascular events.
  
  
  RESULTS
  Amyloidosis patients secondary to FMF showed higher levels of ADMA, CRP and PTX3 and lower FMD as compared with patients with other glomerulopathies. Cardiovascular events (n = 54) were registered during 3 years of follow-up. Increased ADMA levels and lower FMD were observed in patients with cardiovascular risk in both groups, but especially in individuals with amyloidosis.
  
  
  CONCLUSIONS
  Patients with FMF-related amyloidosis have increased CVD event risk, probably related to the high ADMA levels, elevated inflammatory markers and decreased FMD measures observed in these patients.
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Mahmut I
authors.initials: MI
authors.lastname: Yilmaz
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden. Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden. erkandemirkaya@yahoo.com.
authors.forename: Erkan
authors.initials: E
authors.lastname: Demirkaya
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Cengizhan
authors.initials: C
authors.lastname: Acikel
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Saldir
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Servet
authors.initials: S
authors.lastname: Akar
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Tuncer
authors.initials: T
authors.lastname: Cayci
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Mutlu
authors.initials: M
authors.lastname: Saglam
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Hilmi U
authors.initials: HU
authors.lastname: Unal
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Mahmut
authors.initials: M
authors.lastname: Gok
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden. Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Adem
authors.initials: A
authors.lastname: Polat
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Hakkı
authors.initials: H
authors.lastname: Cetinkaya
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Tayfun
authors.initials: T
authors.lastname: Eyileten
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Sebahattin
authors.initials: S
authors.lastname: Sari
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Ali O
authors.initials: AO
authors.lastname: Yildirim
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Alper
authors.initials: A
authors.lastname: Sonmez
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Oguz
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Abdulgaffar
authors.initials: A
authors.lastname: Vural
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden. Department of Nephrology, Department of Pediatric Rheumatology, Gülhane School of Medicine, FMF Arthritis Vasculitis and Orphan Disease Research Center, Institute of Health Sciences, R&D Center, Ankara, Department of Rheumatology, Eylül University, Izmir, Department of Biochemistry, Department of Radiology, Department of Endocrinology, Gülhane School of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Center for Molecular Medicine and Division of Renal Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Juan Jesús
authors.initials: JJ
authors.lastname: Carrero
mesh_terms: D000293:Adolescent; D000328:Adult; D000686:Amyloidosis / Q000150:complications* / Q000503:physiopathology; D001794:Blood Pressure / Q000502:physiology; D002318:Cardiovascular Diseases / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D003430:Cross-Sectional Studies; D004730:Endothelium, Vascular / Q000503:physiopathology*; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D014421:Turkey / Q000453:epidemiology; D014664:Vasodilation / Q000502:physiology*; D055815:Young Adult
keywords: amyloidosis; familiar Mediterranean fever; flow-mediated dilatation; proteinuria; vascular damage