C1q肾病临床研究现状

Advances in clinical research on C1q nephropathy

C1q肾病是一种较为罕见的肾小球肾炎,表现为肾小球系膜区在免疫荧光染色时呈高强度C1q沉积,但是其诊断还需除外系统性红斑狼疮和Ⅰ型膜增生性肾小球肾炎。C1q肾病在光镜下表现是多种多样的,主要有微小病变肾病、局灶节段性肾小球硬化及增生性肾小球肾炎。多表现为持续蛋白尿或者肾病综合征,且多见于男孩。糖皮质激素仍是主要治疗药物之一,免疫抑制剂治疗反应好,但激素抵抗率较高,因此在激素抵抗的情况下,可选择甲泼尼龙冲击或联合免疫抑制剂治疗,预后仍较好。

C1q nephropathy is a rare type of glomerulonephritis manifested as the deposition of C1 q in the glomerular mesangium during immunofluorescent staining. Systemic lupus erythematosus and type I membranoproliferative glomerulonephropathy need to be excluded in the diagnosis of C1 q nephropathy. C1 q nephropathy has various manifestations under a light microscope, mainly including minimal change disease, focal segmental glomerulosclerosis, and proliferative glomerulonephritis. This disease is mainly manifested as persistent proteinuria or nephrotic syndrome and occurs more frequently in boys. Currently, glucocorticoids are mainly used for the treatment of this disease. Patients with C1 q nephropathy show a good response to immunosuppressant treatment, but have a high rate of glucocorticoid resistance. Therefore, in this case, methylprednisolone pulse therapy or a combination with immunosuppressant treatment helps to achieve a good prognosis.