Events - Grand Rounds in Pediatric Nephrology – an ISN-IPNA Sister Centers initiative: Renal amyloidosis presenting as glucocorticoid resistant nephrotic syndrome

Amyloidosis is a rare multi-system disease. In children, amyloid A (AA) amyloidosis (previously known as reactive or secondary amyloidosis) may occur due to chronic inflammation. Pediatric AA amyloidosis mainly  involves the kidneys and presents with proteinuria, hematuria, hypertension and nephrotic syndrome. Therapy for AA amyloidosis is targeted towards control of the underlying inflammatory disease and suppression of serum amyloid protein production. 

In this webinar, we will present a case of renal amyloidosis, successfully treated with IL-6 antibody.  

Learning objectives:

• Differential diagnosis of a child presenting with steroid resistant nephrotic syndrome
• Histological findings in a case with renal amyloidosis and pathogenesis
• Mechanism and role of IL-6 antibody in the treatment of renal amyloidosis

Further reading:

• Renal amyloidosis in children – Pediatr Nephrol. 2011 Aug; 26(8): 1215–1227. Published online 2011 Mar 1.
  DOI: 10.1007/s00467-011-1797-x

• Renal involvement in pediatric rheumatologic diseases – Published online: June 22, 2022. DOI: https://doi.org/10.3339/ckd.22.028

• Successful use of tocilizumab in amyloidosis secondary to systemic juvenile idiopathic arthritis –Rheumatology International volume 40, pages153–159 (2020)