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Etiology, clinical features, and diagnosis of minimal change disease in adults

Author
Alain Meyrier, MD
Section Editors
Richard J Glassock, MD, MACP
Fernando C Fervenza, MD, PhD
Deputy Editor
Albert Q Lam, MD

INTRODUCTION

Minimal change disease, also called nil (Nothing-In-Light microscopy) disease, is a major cause of nephrotic syndrome in both children and adults. Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are often considered together and underlie the idiopathic nephrotic syndrome. Both are podocytopathies, which refers to glomerular diseases that are defined by primary lesions of the podocyte or glomerular epithelial cell. (See 'Pathogenesis' below and "Epidemiology, classification, and pathogenesis of focal segmental glomerulosclerosis", section on 'Pathogenesis'.)

Primary MCD versus primary FSGS — 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]. Both disorders are characterized by diffuse foot process effacement on electron microscopy, the absence of immune deposits, and a severe functional defect in glomerular permselectivity [2]. In addition, both are responsive to glucocorticoid therapy, although the response is much more predictable in MCD than in FSGS. (See 'Pathology' below and "Treatment of primary focal segmental glomerulosclerosis", section on 'Initial immunosuppressive therapy' and "Treatment of minimal change disease in adults", section on 'Immunosuppressive therapy'.)

However, there is suggestive evidence that the pathogenesis of these disorders may be different in at least some patients. As described below, a glomerular permeability factor may be of primary importance in MCD. By contrast, elevated circulating soluble urokinase receptors (suPAR) levels may play a major pathogenetic role in most patients with primary FSGS, including recurrent disease after renal transplantation [3]. In contrast to primary FSGS, serum suPAR levels appear to be normal in MCD and other causes of nephrotic syndrome [3]. However, the role of suPAR in FSGS is controversial, and further studies are needed to assess its practical value for the clinician [4,5]. (See 'Pathogenesis' below and "Epidemiology, classification, and pathogenesis of focal segmental glomerulosclerosis", section on 'suPAR'.)

The pathogenesis, etiology, causes, and diagnosis of MCD in adults will be reviewed here. The treatment of MCD as well as the pathogenesis, diagnosis, and treatment of FSGS are discussed separately. (See "Treatment of minimal change disease in adults" and "Epidemiology, classification, and pathogenesis of focal segmental glomerulosclerosis" and "Treatment of primary focal segmental glomerulosclerosis".)

PATHOLOGY

The glomeruli appear normal on light microscopy in patients with MCD (picture 1) and there are no complement or immunoglobulin deposits on immunofluorescence microscopy. Glomerular size is usually normal by standard methods of light microscopy, although enlarged glomeruli may be observed [6].

                        

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