Korean J Nephrol.  2009 Nov;28(6):653-657.

A Case of Pa-tient with Wegener's Granulomatosis Showing Glomerular Immune Deposition in Kidney Biopsy

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Korea University College of Medicine, Korea. gsong@kumc.or.kr
  • 2Department of Pathology, Korea University College of Medicine, Korea.

Abstract

Rapidly progressive glomerulonephritis (RPGN) in Wegener's granulomatosis patients typically has been characterized by pauci-immune glomerulonephritis (PIGN). In some patients, however, significant amount of glomerular immune deposits was detected and reported that they may have poor prognosis. A 30 year-old-female visited due to the skin rash of both lower extremities, arthralgia and nasal stiffness. She had sinusitis, lung opacity, and proteinuria. Serologic PR-3 ANCA was positive and histologic findings of nasal cavity and lung also showed necrotizing vasculitis and granuloma. Thus we could diagnose Wegener's granulomatosis. However, gross hematuria developed and renal function worsened in spite of treatment with high dose prednisolone and oral cyclophosphamide. Therefore we performed a kidney biopsy. The kidney biopsy showed crescentic glomerulonephritis with Ig A deposition in the mesangium. We experienced a case of Wegener's granulomatosis patient with significant IgA deposition in glomeruli. We report this case with brief review of the literature.

Keyword

Wegener granulomatosis; Immunoglobulin A (IgA); Immune deposition

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Arthralgia
Biopsy
Cyclophosphamide
Exanthema
Glomerulonephritis
Granuloma
Hematuria
Humans
Immunoglobulin A
Kidney
Lower Extremity
Lung
Nasal Cavity
Prednisolone
Prognosis
Proteinuria
Sinusitis
Vasculitis
Wegener Granulomatosis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Immunoglobulin A
Prednisolone
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