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Published ahead of print on September 24, 2009
Clin J Am Soc Nephrol 4: 1747-1753, 2009
© 2009 American Society of Nephrology
doi: 10.2215/CJN.01510209
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Clinical Immunology and Pathology

Dystroglycan in the Diagnosis of FSGS

Giovanna Giannico*, Haichun Yang*, Eric G. Neilson{dagger}, and Agnes B. Fogo*,{dagger}

* Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee; {dagger} Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee

Correspondence: Dr. Agnes B. Fogo,C-3310 MCN, Department of Pathology, Vanderbilt University Medical Center, Nashville, TN 37232-2561. Phone: 615-322-3114; Fax: 615-343-7023; E-mail: agnes.fogo{at}vanderbilt.edu

Background and objectives: {alpha}- and β-dystroglycan (DG), which link the actin cytoskeleton of the podocyte to the glomerular basement membrane, are maintained in FSGS but decreased in minimal change disease (MCD). Fibrosis has been linked to increased fibroblast-specific protein-1 (FSP1) and epithelial–mesenchymal transition. We studied DG, FSP1, and podocyte differentiation in FSGS variants and cases of suspected FSGS.

Design, setting, participants, & measurements: We studied renal biopsies with FSGS, not otherwise specified (NOS), tip lesion, or collapsing variants (COLL), versus secondary FSGS or cases without segmental sclerotic lesions where a diagnosis of MCD versus FSGS could not be established (undefined [UNDEF]) and compared the expression of DG, FSP1, and podocyte Wilms' tumor antigen (WT1).

Results: WT1 is markedly decreased in NOS versus normal and correlates with the extent of sclerosis. {alpha}- and β-DG are maintained in most primary and secondary FSGS cases. In contrast, {alpha}-DG is significantly decreased in UNDEF, supporting a diagnosis of MCD. Furthermore, follow-up shows remission or decreased proteinuria in four of six of these UNDEF cases in response to therapy. Interstitial FSP1 is numerically highest in COLL but is only rarely found in tubules or podocytes in any other forms of FSGS.

Conclusions: We conclude that increased FSP1 may be a marker of the aggressive course of collapsing FSGS. Furthermore, DG staining is a useful adjunct to assist in distinction of FSGS versus MCD in biopsies without defining lesions.


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Dystroglycan in the Molecular Diagnosis of the Podocytopathies
Jeffrey B. Kopp
Clin. J. Am. Soc. Nephrol. 2009 4: 1696-1698. [Full Text] [PDF]



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J. B. Kopp
Dystroglycan in the Molecular Diagnosis of the Podocytopathies
Clin. J. Am. Soc. Nephrol., November 1, 2009; 4(11): 1696 - 1698.
[Full Text] [PDF]


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