J Korean Soc Pediatr Nephrol.  2012 Oct;16(2):132-137.

A Case of Microscopic Polyangiitis Initially Suspected with Henoch-Schonlein Purpura Nephritis

• Im JG
• Moon KC
• Koo JW

Abstract

Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Schonlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Schonlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Schonlein purpura nephritis.

Keyword

Microscopic polyangiitis; p-ANCA; Vasculitis; Henoch-Schonlein purpura

MeSH Terms

Abdominal Pain
Antibodies, Antineutrophil Cytoplasmic
Arthralgia
Biopsy
Child
Cyclophosphamide
Cytoplasm
Exanthema
Glomerulonephritis
Glycosaminoglycans
Hematuria
Humans
Inflammation
Knee
Microscopic Polyangiitis
Nephritis
Outpatients
Proteinuria
Purpura, Schoenlein-Henoch
Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Glycosaminoglycans