Anti-interleukin 1 treatment in secondary amyloidosis associated with autoinflammatory diseases

• DOI: 10.1007/s00467-015-3249-5
• Publication Date: 2015-11-12T11:46:57Z
• Authors: Rezan Topaloglu; Ezgi Deniz Batu; Diclehan Orhan; Seza Ozen; Nesrin Besbas
• Keywords: Male; 0301 basic medicine; Time Factors; Adolescent; Biopsy; Amyloidosis; Kidney; Arthritis, Juvenile; Cryopyrin-Associated Periodic Syndromes; 3. Good health; Interleukin 1 Receptor Antagonist Protein; Proteinuria; 03 medical and health sciences; Treatment Outcome; 0302 clinical medicine; Child, Preschool; Disease Progression; Humans; Immunologic Factors; Female; Kidney Diseases; Child; Retrospective Studies

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.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.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.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.