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Published in: Pediatric Nephrology 4/2016

01-04-2016 | Original Article

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

Authors: Rezan Topaloglu, Ezgi Deniz Batu, Diclehan Orhan, Seza Ozen, Nesrin Besbas

Published in: Pediatric Nephrology | Issue 4/2016

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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.
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Metadata
Title
Anti-interleukin 1 treatment in secondary amyloidosis associated with autoinflammatory diseases
Authors
Rezan Topaloglu
Ezgi Deniz Batu
Diclehan Orhan
Seza Ozen
Nesrin Besbas
Publication date
01-04-2016
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 4/2016
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-015-3249-5

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