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Diagnosis and causes of minimal change disease in adults

INTRODUCTION

Minimal change disease (MCD), or nil (Nothing-In-Light microscopy lesion) disease, is a major cause of nephrotic syndrome in both children and adults. (See "Pathogenesis and diagnosis of focal glomerulosclerosis".) MCD and focal segmental glomerulosclerosis (FSGS) are often considered together and underlie the idiopathic nephrotic syndrome.

There continues to be debate as to whether primary MCD and FSGS are variants of the same disease, or whether they represent separate pathogenetic entities [1]. Findings in support of MCD and FSGS being disease variants include the following:

  • Both are characterized by the absence of immune deposits, diffuse and generalized foot process effacement, and a severe functional defect in glomerular permselectivity [2].
  • Both diseases may be caused by a circulating permeability factor, supporting the hypothesis that MCD and FSGS share a common pathogenesis. However, there is consistent evidence of an immune origin of MCD, whereas the factor, or factors, inducing the podocytopathy that characterizes FSGS has never been identified.
  • The essential prognostic element common to both MCD and FSGS is the response to corticosteroid therapy.

The epidemiology, pathogenesis, causes, and diagnosis of MCD in adults are presented in this topic review. The treatment of MCD as well as the pathogenesis, diagnosis, and treatment of FSGS are presented separately. (See "Treatment of minimal change disease in adults" and "Etiology, clinical manifestations, and diagnosis of nephrotic syndrome in children" and "Pathogenesis and diagnosis of focal glomerulosclerosis" and "Treatment of primary focal glomerulosclerosis".)

PATHOGENESIS

The exact underlying cause of MCD is unclear. Accumulating evidence suggests that systemic T cell dysfunction results in the production of a glomerular permeability factor. This circulating factor directly affects the glomerular capillary wall, resulting in foot process fusion and marked proteinuria.
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