Clinical-pathological Features And Long-term Outcome Of Patients With Primary Focal Segmental Glomerulosclerosis

【Objective】1. To observe the expression of C4d in primary FSGS, and analyze its relationship withclinical indices, pathological indices, response to treatment and prognosis.2. To retrospectively analyze the clinical and pathological features of COLL and CELL,compared with NOS and GTL; and investigate the factors affecting clinical treatment andprognosis of the primary FSGS.【Methods】1. Renal biopsy specimens were collected from patients with primary FSGS, then examedby morphology, immunohistochemistry staining and transmission electron microscope. Toobserve the expression of C4d in primary FSGS, and divided patients into C4d weakpositive/negative group and C4d strong positive group, then comparatively analyze theclinical indices, pathological indices and clinical prognosis of two groups.2. All cases of COLL and CELL primary FSGS were collected in recent8years by thedepartment of pathology, Dongfang Hospital. To analyze the clinical and pathologicalfeatures of them, compared with NOS and GTL; and investigate the related factors toclinical prognosis of patients with primary FSGS.【Results】1. The expression of C4d and the expression of IgM, C3d, C1q, as well as the activityindex, were positively correlated (P<.05), but the clinical features and chronic indexbetween C4d weak positive/negative group and C4d strong positive group had nostatistical difference (P﹥.05).2. The expression of C4d and the remission of patients showed a negative correlation (P<.05); the5-year renal survival rate (89.9%) of C4d weakly positive/negative group washigher than that of C4d strong positive group (66.7%)(P <.05).3. The segmental glomerulosclerosis ratio of COLL and CELL were higher than GTL(P<.05); the global glomerulosclerosis ratio of COLL, as well as the activity index and chronic index, were higher than GTL (P<.05); the average positive score of C4d of COLLwas higher than NOS and GTL (P<.05).4. The remission rate of each group in descending order of GTL (90.6%), CELL (58.3%),NOS (50%) and COLL (37.9%); the remission after treatment was closely associated withpathological subtypes (P<.05). The factors influencing the remission of patients withprimary FSGS contained that SCr at the time of renal biopsy, segmental glomerulosclerosis,global glomerulosclerosis, the average score of TIL, IgM, C3d, C4d, activity index,chronic index, and pathological subtype (P <.05).5. The5-renal survival rate of each group in descending order of GTL (100%), NOS(93.8%), CELL (75.0%), and COLL (58.6%). The factors affecting renal survival ofpatients with primary FSGS contained that SCr at the time of renal biopsy, activity index,chronic index, pathological subtype and the remission after treatment; of which the scoreof chronic index≥10points and COLL are the independent risk factors (P<.05).【Conclusions】1. Complement fragment C4d is not only the effective activity index of FSGS, but also animportant indicator in judging the prognosis of patients with primary FSGS.2. COLL and CELL are the histologic subtypes with obvious clinical pathological features,and have a poor response to treatment and prognosis. The chronic index≥10points andCOLL are the independent risk factors for prognosis of patients with primary FSGS.