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

Journal of Medical Case Reports

Hyun Chul Chung²  Jong Soo Lee²  Jongha Park²  Misung Kim¹  Seung Won Choi²  Chuiyoung Park² 

[1] Department of Pathology, Ulsan University Hospital, 290-3 Jeonha-dong, Dong-gu, Ulsan 682-714, Republic of Korea;Department of Internal Medicine, Ulsan University Hospital, 290-3 Jeonha-dong, Dong-gu, Ulsan 682-714, Republic of Korea

关键词: Membranoproliferative glomerulonephritis;    Valvulopathy;    Arthropathy;    Hypocomplementemic urticarial vasculitis syndrome;   

Others  :  1181001
DOI  :  10.1186/1752-1947-8-352

 received in 2014-05-06, accepted in 2014-08-28,  发布年份 2014

【 摘 要 】

Introduction

Hypocomplementemic 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 presentation

A 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.

Conclusions

We 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.

【 授权许可】

   
2014 Park et al.; licensee BioMed Central Ltd.

【 图 表 】

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