Clinicopathologic Analysis Of Primary Focal Segmental Glomerulosclerosis

Objective: To study the clinical manifestations of primary FSGS, and to study the associations between different pathologic types and manifestations, and to improve the understanding of FSGS in clinician level, and to provide guidance for future clinical work.Methods: One hundred and fifty-two cases of FSGS proven by renal biopsy were retrieved from the first affiliated hospital of Guangxi medical university during November 2002 to November 2010, the clinical manifestations and pathology characteristic were studied retrospectively, and the associations between different pathologic types and manifestations were explored.Results: 1. The average age of the 152 patients was 35.1±15.0 years, 126 of the 152 patients were less than 50 years (82.9%), 10 patients were more than 60 years (6.6%). The ratio of male to female was 1.76:1. 2.The main clinical features were as follows: 95.4% patients had proteinuria , 66.4% cases presented with nephritic-range proteinuria, (47.7%) nearly half of patients had microscopic hematuria, and 2.6%patients had gross hematuria, 42.8%patients had hypertension, 21.7%patients had urine sugar positive, indicating proximal tubular dysfunction, 30.3% patients suffered from renal insufficiency. Reduction of serum IgG was found in 108 of the 124 patients(87.1%). 3. Pathology classification, and different pathological types and clinical relationship: The frequency of not otherwise variant FSGS in the primary FSGS was 51.3%, tip variant FSGS 27.6%, perihilar variant FSGS 4.5%, cellular variant FSGS3.9%,and Collapsing FSGS was not found. The level of proteinuria in tip variant was much higher than that in not otherwise and perihilar variants(P<0.05). The incidence of nephritic-range proteinuria in tip variant was much higher than that in the perihilar variant(P<0.05). the level of serum albumin in tip variant was higher than that in not otherwise variant and the level of serum cholesterol in tip variant was higher than that in not otherwise variant(P<0.05). The activity score of the tip variant was higher than that of the not otherwise and perihilar variant(sP<0.05). The chronicity score of the perihilar variant was much higher than that of the tip variant(P<0.05).Conclusions: 1.The main clinical manifestation of primary FSGS presents with nephrotic syndrome. Most of patients often are accompanied by microscopic hematuria, and a few patients present with macroscopic hematuria.Hypertension tubulointerstitial lesions, renal insufficiency and reduction of serum IgG are frequently morphologic pattern seen seen. 2.The not otherwise variant is the commonest morphologic pattern seen in primary FSGS. 3.The tip variant has more urine protein, higher level of serum cholesterol and lower level of serum albumin than the not otherwise variant .The tip variant has more urine protein and higher incidence of nephrotic syndrome than the perihilar. 4.The tip variant is the pattern associated with active lesions, The perihilar variant is the pattern associated with chronic lesions.