Diagnosis of primary FSGS is confirmed by renal biopsy which demonstrates glomerular scarring in a focal (involving some but not all glomeruli) and segmental (involving portions of any single glomeruli) pattern. Primary FSGS is a progressive disease which has a profound impact on quality of life, morbidity and mortality. Secondary causes include the following, in approximate order of frequency: diabetes mellitus, lupus erythematosus, amyloidosis and paraproteinemias, viral infections (eg, hepatitis B, hepatitis C, human immunodeficiency virus ), and preeclampsia. Primary causes of nephrotic syndrome include the following, in approximate order of frequency: minimal-change nephropathy, focal glomerulosclerosis, membranous nephropathy, and hereditary nephropathies. In all cases, injury to glomeruli is an essential feature. Nephrotic syndrome can be primary, being a disease specific to the kidneys, or it can be secondary, being a renal manifestation of a systemic general illness. Notably, nephrotic syndrome may affect adults and children, of both sexes and of any race. Nephrotic syndrome can also result from systemic diseases that affect other organs in addition to the kidneys, such as diabetes, amyloidosis, and lupus erythematosus. There are many specific causes of nephrotic syndrome, including kidney diseases such as minimal-change nephropathy, focal glomerulosclerosis, and membranous nephropathy. The majority of FSGS cases are characterized by progressive renal fibrosis and steady deterioration in kidney function.įocal segmental glomerulosclerosis classically presents with nephrotic syndrome: proteinuria ≥3.0 g/24 hours associated with signs/symptoms including hypoalbuminemia, edema and hyperlipidemia. Primary FSGS has a distinctive histopathological appearance, characterized by hyalinization and sclerosis of a portion of the glomerular tuft, minimal deposition of immune complexes, and effacement of visceral epithelial cell (podocyte) foot processes. Primary (or idiopathic) focal segmental glomerulosclerosis (herein after referred to as “primary FSGS”) is a kidney disease diagnosed based on clinicopathological finding. FSGS is not a single disease rather, it is a heterogeneous clinicopathological process identified in renal biopsies that are generally performed in patients with proteinuria or nephrotic syndrome. BACKGROUND OF THE INVENTIONįocal segmental glomerulosclerosis (FSGS) is a clinical entity that impacts renal function through progressive fibrosis of the glomerulus. In particular, the method relates to administering a pharmaceutical formulation comprising a therapeutically effective amount of fresolimumab, to a patient having primary FSGS, such as a patient having a genetic variant associated with an increased risk in having or developing primary FSGS, for example, an APOL1 genetic variant, so that the patient may derive a benefit therefrom. The present invention relates to a method of treating primary focal segmental glomerulosclerosis (primary FSGS) and/or improving one or more symptoms associated with primary FSGS, comprising administering a TGFβ antagonist to a patient in need thereof. 8,591,901, in their entirety, are also each incorporated herein by reference. The foregoing related applications, in their entirety, are incorporated herein by reference. 17, 2016, which claims the benefit of priority from U.S.
This application is a National Stage under 35 U.S.C.
0 kommentar(er)
