J Korean Soc Pediatr Nephrol.  2005 Oct;9(2):231-236.

A Case of ANCA-associated Pauci-immune Crescentic Glomerulonephritis in Juvenile Rheumatoid Arthritis

• Hwang YS
• Rhie YJ
• Ahn SY
• Kim DS
• Lee JS
• Jeong HJ

Abstract

Juvenile rheumatoid arthritis(JRA) is the most common major connective tissue disease in children. Renal involvement in JRA is rare. Among the renal lesions that have been reported in JRA, amyloidosis and drug-induced nephropathy are the most common. Crescentic glomerulonephritis in JRA has rarely been reported. We report a case of ANCA-associated pauci-immune crescentic glomerulonephritis in JRA. The patient was a 15-year old boy with a 3-year history of JRA. He presented with gross hematuria, proteinuria, positive p-ANCA and elevation of BUN and creatinine. Pathologic findings revealed focal necrotizing and crescentic glomerulonephritis. There were no significant immunoglobulin or complement deposits. His renal function recovered after intravenous methylprednisolone pulse therapy and oral steroid use. In Korea, this is the first reported case of pauci-immune crescentic glomerulonephritis in JRA.

Keyword

Juvenile rheumatoid arthritis; ANCA; Crescentic glomerulonephritis

MeSH Terms

Adolescent
Amyloidosis
Antibodies, Antineutrophil Cytoplasmic
Arthritis, Juvenile*
Child
Complement System Proteins
Connective Tissue Diseases
Creatinine
Glomerulonephritis*
Hematuria
Humans
Immunoglobulins
Korea
Male
Methylprednisolone
Proteinuria
Antibodies, Antineutrophil Cytoplasmic
Complement System Proteins
Creatinine
Immunoglobulins
Methylprednisolone