Presentation, Etiology and RiskFactors in Patients with Focal and SegmentalGlomerulosclerosis.

INTRODUCTION : Focal and segmental glomerulosclerosis (FSGS) is defined as a clinicalpathologic syndrome manifesting proteinuria, usually of nephrotic range, associated with lesions of focal and segmental glomerular sclerosis and foot process effacement. Although hyaline insudation is common, the condition lacks glomerular immune complex deposits. Early in the disease process, the pattern of glomerular sclerosis is focal, involving a subset of glomeruli, and segmental, involving a portion of the glomerular tuft. As the disease progresses, a more diffuse and global pattern of sclerosis evolves. Alterations of the podocyte cytoarchitecture constitute the major ultrastructural findings. FSGS came to be viewed as a distinct pathologic entity by 1970. In recent years it has become clear that FSGS is seen more usefully as a clinicopathologic syndrome, comprising diverse distinct diseases with different etiologies. These include genetic mutation (likely showing both Mendelian and non-Mendelian modes of inheritance), viral infection, relative overabundance of a pathogenic plasma factor, and hyperfiltration injury. Doubtless, there are other disease entities within the FSGS syndrome that remain undefined. Among the biopsy proven renal disease in adults, focal and segmental glomerulosclerosis is the commonest histological category in all age groups (16.8%). Although the pathogenesis of primary focal and segmental glomerulosclerosis (FSGS) is unknown, it is one of the most common causes of primary glomerular disease that terminate in end stage renal disease (ESRD). In most series, the 10-year survival is between 40-60%. Various studies have shown that clinical, biochemical and pathological indicators can help to predict renal failure in a patient with FSGS. These are age at the time of presentation, gender, amount of proteinuria, hypertension, renal dysfunction, percentage of sclerosed glomeruli and interstitial fibrosis, location of segmental sclerosis and therapeutic response to steroids. Whether these prognostic factors and survival rate mentioned in the western literature are relevant to Indian population is unknown. In the present study, we analysed the clinical, laboratory, and histopathological data of adult patients with biopsy proven FSGS over 10 years to identify risk factors at diagnosis associated with progression and to determine survival rates. CONCLUSIONS : 1. Clinical and pathological indicators help to predict renal failure in adult primary FSGS at the time of presentation. 2. Independent predictors of disease progression are a. amount of proteinuria b. degree of interstitial fibrosis 3. Independent predictors of development to ESRD in adult primary FSGS are a. nephrotic range proteinuria b. low serum albumin c. degree of interstitial fibrosis 4. Renal failure at presentation is not a predictor of progression could be because of high risk nephrotics presented earlier as swelling appeared even when the renal function was preserved. 5. This study predict the probability of renal survival in adult primary FSGS at presentation and therefore identify high risk group.