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Minimal-change nephropathy and focal segmental glomerulosclerosis 

Minimal-change nephropathy and focal segmental glomerulosclerosis

Chapter:
Minimal-change nephropathy and focal segmental glomerulosclerosis
Author(s):

Dwomoa Adu

DOI:
10.1093/med/9780199204854.003.210803

May 25, 2011: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 28 April 2017

Minimal-change nephrotic syndrome

Minimal-change nephrotic syndrome (MCNS) is an immune-mediated condition, usually of unknown cause, but which can sometimes be associated with Hodgkin’s disease or the use of nonsteroidal anti-inflammatory drugs. On light microscopy the glomeruli appear normal or small, and on electron microscopy there is effacement of epithelial-cell foot processes over the outer surface of the glomerular basement membrane. MCNS is the cause of about 80% of cases of nephrotic syndrome in children and 20% in adults.

Management and prognosis—treatment in adults is with prednisolone at an initial dose of 60 mg/day (then tapering), with 75% responding by 6 months. Up to 60% of patients who go into a remission have a relapse, and about 40% have frequent relapses, and in these patients treatment with cyclophosphamide induces a sustained remission in 60% over a 5-year period. Ciclosporin is also of benefit in frequent relapsers, but most patients relapse when this is discontinued. Just over 5% of patients remain nephrotic in the long term. Progression to renal failure is not expected and would call the diagnosis of MCNS into question.

Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is not a specific disease entity but a histological lesion, often of unknown aetiology, which is characterized by segmental areas of glomerular sclerosis. It may be: (1) primary—which is by definition of unknown cause, but in about 30% of cases is associated with a circulating protein factor that causes an increase in glomerular permeability; or (2) secondary—the end product of a variety of pathological processes including glomerular hyperfiltration, healed glomerulonephritis, viral infection (HIV), and genetic mutation. Based on the site of the lesions and other histological features the primary condition can be divided into five variants: (1) perihilar; (2) glomerular tip; (3) collapsing variant; (4) cellular variant; and (5) ‘not otherwise specified’, when the other variants have been excluded. Most patients with FSGS present with nephrotic syndrome (FSGS is the diagnosis in 20% of adults with nephrotic syndrome), some with persistent proteinuria, and a few have haematuria as well as proteinuria.

Management and prognosis—patients with primary FSGS and nephrotic syndrome should be treated with prednisolone for 6 months (initially 60 mg/day, then tapering), and those who are resistant should receive ciclosporin for 26 to 52 weeks. Those who achieve a complete remission have a 5-year survival off dialysis of over 90%, as compared with about 50% of those who do not achieve remission. Patients with the glomerular tip lesion respond best; those with classic FSGS (‘not otherwise specified’) have an intermediate response; and those with collapsing FSGS have the worst prognosis. The nephrotic syndrome recurs—often within days—after renal transplantation in 20 to 40% of patients with primary FSGS, leading to graft failure in approximately 50% of cases.

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