Lioufas Nicole, Finlay Moira, Barbour Thomas
Department of Nephrology, The Royal Melbourne Hospital, Victoria, Australia.
Department of Anatomical Pathology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia.
Nephrology (Carlton). 2017 Feb;22 Suppl 1:36-39. doi: 10.1111/nep.12939.
In C3 glomerulopathy, uncontrolled complement C3 activation via the alternative pathway results in glomerular C3 deposition and, in many cases, progressive renal failure. Despite advances in understanding of C3G pathogenesis over the last few years, there are no proven treatments. We describe a patient in whom C3 glomerulopathy was associated with renal impairment and elevated serum free kappa light chains. An initial response to corticosteroids was followed by relapse once steroids were weaned, prompting use of mycophenolate mofetil to maintain remission. We discuss some of the diagnostic and therapeutic issues surrounding C3G, including in the setting of monoclonal gammopathy.
在C3肾小球病中，通过替代途径的补体C3不受控制的激活导致肾小球C3沉积，并且在许多情况下会导致进行性肾衰竭。尽管在过去几年中对C3G发病机制的理解有所进展，但尚无经过验证的治疗方法。我们描述了一名C3肾小球病患者，其伴有肾功能损害和血清游离κ轻链升高。最初对皮质类固醇有反应，但一旦停用类固醇就复发，促使使用霉酚酸酯来维持缓解。我们讨论了围绕C3G的一些诊断和治疗问题，包括在单克隆丙种球蛋白病的情况下。
