J Korean Rheum Assoc.  2010 Sep;17(3):321-325.

Microscopic Polyangiitis Presenting as Membranoproliferative Glomerulonephritis

• Jeong MJ
• Kim TW
• Im JY
• Jeong JS
• Park MR
• Kim TW
• Lee CW

Abstract

Microscopic polyangiitis (MPA) is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. The kidney is the most often affected organ in the majority of patients with MPA, and renal manifestations are usually the first symptoms. Glomerular capillaries are affected most often, resulting in necrotizing glomerulonephritis, usually in a crescent formation, with no or few immune deposits able to be demonstrated at the sites of vasculitis and glomerulonephritis. We report a case of microscopic polyangiitis in both legs with pitting edema in a 50-year-old female. Laboratory findings showed hematuria, proteinuria, and a positive peripheral antineutrophil cytoplasmic antibody. A renal biopsy revealed pauci-immune splitting and necrotizing capillary loop walls necrotizing vasculitis and membranoproliferative glomerulonephritis (MPGN). With a diagnosis of MPA, she has been managed with high dose steroid and cyclophosphamide. To our knowledge, this is the first reported case of MPA with MPGN.

Keyword

Microscopic polyangiitis; Membranoproliferative glomerulonephritis

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Biopsy
Capillaries
Cyclophosphamide
Edema
Female
Glomerulonephritis
Glomerulonephritis, Membranoproliferative
Glycosaminoglycans
Hematuria
Humans
Inflammation
Kidney
Leg
Microscopic Polyangiitis
Middle Aged
Proteinuria
Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Glycosaminoglycans