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    Standard immunosuppressive therapy of immune-mediated glomerular diseases

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    Glomerulonephritis (GN) accounts for 10%-20% of the total incident cases of end stage renal disease (ESRD), and is the third most common cause of ESRD after diabetes and hypertension in western countries. The pathogenesis of glomerulonephritis is prevalently immune mediated: humoral and cell-mediated immunity are involved, although the rationale for an etiological treatment is still lacking. In the last forty years, empirical treatment based upon the use of corticosteroids and/or immunosuppressive drugs have obtained excellent results in improving survival of both the patient and the kidney. Almost 95% of children affected by minimal change disease (MCD) achieve remission of proteinuria within 4 to 8. weeks of prednisone administration. In adults with focal segmental glomerulosclerosis (FSGS), prednisone induces complete or partial remission in the majority of patients, but a longer period of steroid treatment or the combination of calcineurin inhibitors or cytotoxic drugs can be needed. A percentage of 65%-70% of patients with idiopathic membranous nephropathy (MN) reach complete or partial remission with a 6-month course of therapy alternating glucocorticoids with alkylating agents. Glucocorticoids plus cyclophosphamide, and, on occasion, plasmapheresis are effective in 70%-90% of patients with ANCA-associated vasculitis (AAV). Fifty percent of responders relapse within the 3-5. years and currently, the mortality of AAV at 1. year exceeds 15%.This article is aimed to analyze the risk-to-benefit balance of steroids and conventional immunosuppressive regimens, focusing, for a sake of brevity, on idiopathic nephrotic syndrome (INS) and ANCA associated vasculitis

    FSGS: from pathogenesis to the histological lesion

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    Focal and segmental glomerulosclerosis (FSGS) is a generic term that in the current terminology can be appropriate for identifying two conditions. First, a morphological pattern of injury characterized by the occlusion of a single or group of glomerular capillary loops by sclerotic material, indicating a precise histological lesion but with a wide range of etiological interpretations. Second, a pauci-immune renal disease called idiopathic or primary FSGS, which is a podocytopathy triggered by an endogenous cytotoxin that recognizes the podocyte as its sole target. Based on the current and past literature, we do not possess practical tools to easily provide a precise classification of an FSGS lesion, although some clues may be of help in everyday clinical practice. Reactive forms, genetic forms, adaptive forms, forms associated with a deregulation of the proliferation and forms secondary to local glomerular inflammation are the etiological classes known to be associated with the development of an FSGS lesion. However, diagnosing each single case based on clinical, serological and histological criteria is still far from easy and mostly depends on the experience of the renal team, which should involve skilled nephrologists and pathologists
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