title: AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis.
abstract: We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.
authors.affiliation: Division of Pediatric Nephrology, PGIMER & Associated Dr. RML Hospital, New Delhi, India.
authors.forename: Abhijeet
authors.initials: A
authors.lastname: Saha
authors.forename: Yogiraj
authors.initials: Y
authors.lastname: Chopra
authors.forename: Jason D
authors.initials: JD
authors.lastname: Theis
authors.forename: Julie A
authors.initials: JA
authors.lastname: Vrana
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
mesh_terms: D000686:Amyloidosis / Q000150:complications*; D001171:Arthritis, Juvenile / Q000150:complications*; D002648:Child; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology*; D000685:Serum Amyloid A Protein
chemical_list: D000685:Serum Amyloid A Protein
keywords: AA amyloid; mass spectrometry; proteomics; systemic-onset juvenile idiopathic arthritis