Clinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a case report and literature review

BMC Nephrology
Songkiat ChantaroghSuchin Worawichawong

Abstract

Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis, this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies have revealed that common heterozygous or homozygous mutations in the MEFV gene are associated with systemic-onset JIA (SJIA). We herein report a case involving a 19-year-old female patient with difficult-to-control SJIA. She developed progressive proteinuria without clinical signs or symptoms of edema. Renal amyloidosis was diagnosed by renal pathologic examination, which demonstrated deposition of eosinophilic amorphous material in the interlobular arteries, arterioles, and interstitium. Electron microscopy showed fibrillary material deposits with a diameter of 8 to 10 nm. A heterozygous E148Q mutation in the MEFV gene was identified. Conventional disease-modifying anti-rheumatic drugs and etanercept had been used to treat the SJIA, but the disease could not be controlled. Therefore, we decided to start tocilizumab to control the disease activity. However, the patient was unable to receive a standard dose of tocilizumab in the early period of treatment because of socioeconomic limitations...Continue Reading

References

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Citations

Dec 16, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Seza Ozen
Sep 7, 2019·Seminars in Arthritis and Rheumatism·M DelplanqueS Georgin-Lavialle
Jan 12, 2020·La Revue de médecine interne·M FauterY Jamilloux

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Methods Mentioned

BETA
urine collection
biopsy
polarized microscopy
electron microscopy
ESR

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