[Minimal change disease and focal segmental glomerulosclerosis]

Internist (Berl). 2019 May;60(5):450-457. doi: 10.1007/s00108-019-0590-y.
[Article in German]

Abstract

Minimal change disease (MCD) or minimal change glomerulonephritis and focal segmental glomerulosclerosis (FSGS) are the two major causes of nephrotic syndrome in children and young adults. Both disease entities resemble each other and can sometimes only be discriminated on the basis of their clinical courses. MCD and FSGS display two classical examples that share a common pathophysiology in which the glomerular podocyte and the cytoskeleton of its foot processes play important roles. Therefore, the term "podocytopathy" was introduced for both diseases. In this article, we compare their differences and similarities, and summarized new data on pathophysiology and treatment. In adults, only a renal biopsy including electron microscopy allows for the discrimination of MCD and FSGS and other differential diagnoses. The identification of a primary or secondary form of the disease is based on the clinical course. Data from studies on the treatment are sparse; hence, treatment is still based on high-dose steroids followed by additional immunosuppressive agents. In secondary forms, treatment of the underlying disease is elementary.

Keywords: Biopsy, kidney; Immunosuppression; Nephrotic syndrome; Podocytes; Steroids.

Publication types

  • Review

MeSH terms

  • Child
  • Glomerulosclerosis, Focal Segmental / complications
  • Glomerulosclerosis, Focal Segmental / diagnosis*
  • Glomerulosclerosis, Focal Segmental / drug therapy
  • Humans
  • Immunohistochemistry
  • Immunosuppressive Agents / therapeutic use
  • Kidney / pathology*
  • Nephrosis, Lipoid / complications
  • Nephrosis, Lipoid / diagnosis*
  • Nephrosis, Lipoid / drug therapy
  • Nephrotic Syndrome / complications
  • Nephrotic Syndrome / diagnosis*
  • Nephrotic Syndrome / drug therapy
  • Steroids / therapeutic use
  • Young Adult

Substances

  • Immunosuppressive Agents
  • Steroids