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http://dx.doi.org/10.3339/jkspn.2012.16.2.132

A Case of Microscopic Polyangiitis Initially Suspected with Henoch-Sch$\ddot{o}$nlein Purpura Nephritis  

Im, Jong Geun (Department of Pediatrics, Sanggye Paik Hospital, Inje University College of Medicine)
Moon, Kyung Chul (Department of Pathology, Seoul National University College of Medicine)
Koo, Ja Wook (Department of Pediatrics, Sanggye Paik Hospital, Inje University College of Medicine)
Publication Information
Childhood Kidney Diseases / v.16, no.2, 2012 , pp. 132-137 More about this Journal
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-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein 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-Sch$\ddot{o}$nlein purpura nephritis.
Keywords
Microscopic polyangiitis; p-ANCA; Vasculitis; Henoch-Sch$\ddot{o}$nlein purpura;
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1 Peco AA, Bonaci NB, Basta JG, Kostic M, Markovic LJ, Nikolic M, et al. Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol 2006;21:46-53.   DOI   ScienceOn
2 Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999; 42:421-30.   DOI   ScienceOn
3 Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med 1985;56:467-83.
4 Uhm WS. ANCA associated vasculitis. J Korean Rheum Assoc 2010;17:108-32.   DOI   ScienceOn
5 Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36-44.   DOI   ScienceOn
6 Davson J, Ball J, Platt R. The kidney in periarteritis nodosa. Q J Med 1948;17:175-202.
7 Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-92.   DOI   ScienceOn
8 Seo P, Stone JH, The antineutrophil cytoplasmic antibodyassociated vasculitides. Am J Med 2004;117:39-50.
9 Lee JE, Chae YS, Lee OK, Lee DY. A Case of microscopic polyangitis presented as pulmonary hemorrhage and rapidly progressive glomerulonephritis. J Korean Pediatr Soc 1999; 42:274-8.
10 Kwon HS, Lee YM, Kim JH, Kim PK, Kang HY, Hong SW, et al. A Case of microscopic polyangiitis with pulmonary hemorrhage and rapidly progressive glomerulonephritis. J Korean Soc Pediatric Nephrol 2001;5:213-8.
11 Hattori M, Kurayama H, Koitabashi Y. Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol 2001;12:1493-500.
12 Jeong MJ, Kim TW, Im JY, Jeong JS, Park MR, Kim TW, et al. Microscopic polyangiitis presenting as membranoproliferative glomerulonephritis. J Korean Rheum Assoc 2010;17:321-5.   DOI
13 Pusey CD, Rees AJ, Evans DJ, Peters DK, Lockwood CM. Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 1991;40:757-63.   DOI   ScienceOn
14 Akioka Y, Hattori M, Kawaguchi H, Ito K. A case of chronic relapsing ANCA-associated microscopic polyangiitis successfully treated with plasma exchange. Ther Apher Dial 2004;8: 223-6.   DOI   ScienceOn
15 Yuksel S, Yalcinkaya F, Ozcakar ZB, Acar B, Tulunay O, Ekim M. A girl with microscopic polyangiitis: an unexpected clinical course with long-term follow-up. Pediatr Nephrol 2005;20: 694-5.   DOI   ScienceOn
16 Jindal G, Cruz SD, Punia RP, Kaur R. Refractory anemia as a presenting feature of microscopic polyangiitis: a rare vasculitis in children. Indian J Pediatr 2011;78:1287-9.   DOI   ScienceOn
17 Mansi IA, Opran A, Rosner F. ANCA-associated small vessel vasculitis. Am Fam Physicial 2002;65:1615-20.
18 Maejima H, Shirai K, Shimamura Y, Harada H, Eto H. Microscopic polyangiitis presenting urticarial erythema and Henoch- Schonlein purpura: two case reports. J Dermatol 2004;31: 655-60.   DOI
19 Nagasaka T, Miyamoto J, Ishibashi M, Chen KR. MPO-ANCAand IgA-positive systemic vasculitis: a possibly overlapping syndrome of microscopic polyangiitis and Henoch-Schoenlein purpura. J Cutan Pathol 2009;36:871-7.   DOI   ScienceOn
20 Savage CO. ANCA-associated renal vasculitis. Kidney Int 2001;460:1614-27.