Immunologic Changes Implicated in the Pathogenesis of Focal Segmental Glomerulosclerosis

Andreas Kronbichler, Johannes Leierer, Jun Oh, Björn Meijers, Jaeil Shin

Research output: Contribution to journalReview article

8 Citations (Scopus)

Abstract

Focal segmental glomerulosclerosis is a histological pattern on renal biopsy caused by diverse mechanisms. In its primary form, a circulatory factor is implicated in disease onset and recurrence. The natural history of primary FSGS is unpredictable, since some patients are unresponsive towards immunosuppressive measures. Immunologic changes, leading to a proinflammatory or profibrotic milieu, have been implicated in disease progression, namely, glomerular scarring, eventually leading to end-stage renal disease. Among these, interleukin-1β, tumor-necrosis factor-α (TNF-α), and transforming growth factor-1 (TGF-β1) have emerged as important factors. Translating these findings into clinical practice dampened the enthusiasm, since both TNF-α and TGF-β1 blockade failed to achieve significant control of the disease. More recently, a role of the complement system has been demonstrated which in fact may be another attractive target in clinical practice. Rituximab, blocking CD20-bearing cells, demonstrated conflicting data regarding efficacy in FSGS. Finally, the T-cell costimulating molecule B7-1 (CD80) is implicated in development of proteinuria in general. Blockade of this target demonstrated significant benefits in a small cohort of resistant patients. Taken together, this review focuses on immunology of FSGS, attributable to either the disease or progression, and discusses novel therapeutic approaches aiming at targeting immunologic factors.

Original languageEnglish
Article number2150451
JournalBioMed Research International
Volume2016
DOIs
Publication statusPublished - 2016 Jan 1

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Focal Segmental Glomerulosclerosis
Transforming Growth Factors
Disease Progression
Tumor Necrosis Factor-alpha
B7 Antigens
Immunologic Factors
Immunosuppressive Agents
Allergy and Immunology
Natural History
Interleukin-1
Proteinuria
Chronic Kidney Failure
Cicatrix
T-Lymphocytes
Kidney
Biopsy
Recurrence
Bearings (structural)
Immunology
T-cells

All Science Journal Classification (ASJC) codes

  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Microbiology(all)

Cite this

Kronbichler, Andreas ; Leierer, Johannes ; Oh, Jun ; Meijers, Björn ; Shin, Jaeil. / Immunologic Changes Implicated in the Pathogenesis of Focal Segmental Glomerulosclerosis. In: BioMed Research International. 2016 ; Vol. 2016.
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Immunologic Changes Implicated in the Pathogenesis of Focal Segmental Glomerulosclerosis. / Kronbichler, Andreas; Leierer, Johannes; Oh, Jun; Meijers, Björn; Shin, Jaeil.

In: BioMed Research International, Vol. 2016, 2150451, 01.01.2016.

Research output: Contribution to journalReview article

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