title: Anti-interleukin 1 treatment in secondary amyloidosis associated with autoinflammatory diseases.
abstract: BACKGROUND
  Amyloidosis may complicate autoinflammatory diseases (AID). We aimed to evaluate the renal biopsy findings, and clinical and laboratory parameters in patients with AID-associated amyloidosis who have responded to anti-interleukin 1(IL1) treatment.
  
  
  METHODS
  Two children with systemic juvenile idiopathic arthritis and one with cryopyrin-associated periodic syndrome diagnosed as having reactive amyloidosis were treated with anti-IL1 drugs. The renal histopathological findings at the time of diagnosis of amyloidosis and after the onset of anti-IL1 were evaluated according to the amyloid scoring/grading system.
  
  
  RESULTS
  The median age of disease onset and diagnosis of amyloidosis were 3 and 12 years, respectively. Anakinra was started in all; however, anakinra caused a local cutaneous reaction in one, thus canakinumab was commenced. Proteinuria improved in all. Control renal biopsies were performed a median of 3 years after the first biopsies. The renal amyloid prognostic score did not improve in patient 1, and progressed in patients 2 and 3. The renal amyloid grade progressed in patient 2.
  
  
  CONCLUSIONS
  This is the first series demonstrating progression of renal tissue damage after the improvement of proteinuria with anti-IL 1 in AID-associated amyloidosis. Anti-IL1 drugs are important to prevent further amyloid accumulation; however, new treatment strategies are needed to target the amyloid deposits.
authors.affiliation: Department of Pediatrics, Division of Nephrology, Hacettepe University Faculty of Medicine, Ankara, 06100, Turkey. rezantopaloglu@hacettepe.edu.tr.
authors.forename: Rezan
authors.initials: R
authors.lastname: Topaloglu
authors.affiliation: Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Ezgi Deniz
authors.initials: ED
authors.lastname: Batu
authors.affiliation: Department of Pediatrics, Division of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Diclehan
authors.initials: D
authors.lastname: Orhan
authors.affiliation: Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Department of Pediatrics, Division of Nephrology, Hacettepe University Faculty of Medicine, Ankara, 06100, Turkey.
authors.forename: Nesrin
authors.initials: N
authors.lastname: Besbas
mesh_terms: D000293:Adolescent; D000686:Amyloidosis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D001171:Arthritis, Juvenile / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D056587:Cryopyrin-Associated Periodic Syndromes / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D018450:Disease Progression; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects / Q000627:therapeutic use*; D053590:Interleukin 1 Receptor Antagonist Protein / Q000009:adverse effects / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D007674:Kidney Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D011507:Proteinuria / Q000188:drug therapy / Q000276:immunology; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D053590:Interleukin 1 Receptor Antagonist Protein
keywords: Anakinra; Anti-interleukin 1; Autoinflammatory disease; Proteinuria; Renal amyloidosis