C3肾小球病临床表现和病理改变的异质性

Heterogeneity of the clinical manifestations and pathology features in C3 glomerulopathy

C3肾小球病是由于补体旁路途径异常活化介导的疾病。由于致病机制的多样性和多重性,C3肾小球病的临床表现与病理改变具有异质性,临床可以表现为尿检异常、高血压、肉眼血尿、肾病综合征、肾炎综合征、肾功能不全等,病理改变可以表现为膜增生性肾小球肾炎、系膜增生、新月体形成、局灶节段性坏死、弥漫增生和渗出病变等,预后也不均一。本文对C3肾小球病的临床表现及病理特征进行综述。

C3 glomerulopathy is a kind of glomerular diseases mediated by abnormal activation of alternative complement pathway. As diversity and multiplicity of pathogenic mechanism, heterogeneity exists in the clinical manifestation and pathological features of C3 glomerulopathy. The clinical manifestation of the disease may be shown as abnormality in urine, hypertension, hematuria, nephrotic syndrome, nephritic syndrome, renal insufficiency, etc. Membranoproliferative glomerulonephritis, mesangial proliferation, crescent formation, focal segmental necrosis, diffuse hyperplasia and exudative lesions, etc may be found in renal biopsies. Also, the prognosis of C3 glomerulopathy is not uniform. The clinical manifestations and pathological features of C3 glomerulopathy were reviewed in the present paper.