Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case reportReport as inadecuate




Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report - Download this document for free, or read online. Document in PDF available to download.

Journal of Medical Case Reports

, 8:352

First Online: 22 October 2014Received: 06 May 2014Accepted: 28 August 2014DOI: 10.1186-1752-1947-8-352

Cite this article as: Park, C., Choi, S.W., Kim, M. et al. J Med Case Reports 2014 8: 352. doi:10.1186-1752-1947-8-352

Abstract

IntroductionHypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud’s arthropathy, and valvular heart disease have been reported.

Case presentationA 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil.

ConclusionsWe describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

KeywordsHypocomplementemic urticarial vasculitis syndrome Arthropathy Valvulopathy Membranoproliferative glomerulonephritis AbbreviationsANAAntinuclear antibodies

anti-CCPAnti-cyclic citrullinated peptide antibody

ANCAAntineutrophil cytoplasmic antibody

BUNBlood urea nitrogen

CTComputed tomography

ESRErythrocyte sedimentation rate

HUVSHypocomplementemic urticarial vasculitis syndrome

MMFMycophenolate mofetil

MPGNMembranoproliferative glomerulonephritis

SLESystemic lupus erythematosus.

Electronic supplementary materialThe online version of this article doi:10.1186-1752-1947-8-352 contains supplementary material, which is available to authorized users.

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Author: Chuiyoung Park - Seung Won Choi - Misung Kim - Jongha Park - Jong Soo Lee - Hyun Chul Chung

Source: https://link.springer.com/



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