NDT Plus Advance Access originally published online on January 17, 2008
NDT Plus 2008 1(3):187; doi:10.1093/ndtplus/sfm019
© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.For Permissions, please e-mail: journals.permissions@oxfordjournals.org
Intramembranous electron-dense deposits in a nephrotic patient with mixed connective tissue disease: unusual response to corticosteroid therapy
Itaru Ebihara1,
Joichi Usui2,
Kouichi Hirayama3,
Masaki Kobayashi3 and
Kunihiro Yamagata2
1 Department of Nephrology, Mito Saiseikai General Hospital, Mito
2 Department of Nephrology, Institute of Clinical Medicine, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba
3 Department of Nephrology, Tokyo Medical University Kasumigaura Hospital, Ami, Ibaraki, Japan
Correspondence: Itaru Ebihara, Department of Nephrology, Mito Saiseikai General Hospital, 3-3-10 Futabadai, Mito, Ibaraki, 311-4198 Japan; E-mail: itaruebi{at}yahoo.co.jp
Received for publication October 27, 2007. Accepted for publication November 7, 2007.
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Case
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A 56-year-old female patient, who had had minor change nephrotic
syndrome (MCNS), was referred to us because of a fever and a
massive proteinuria. Six years before, she had first noticed
leg oedema, leading to the detection of nephrotic-range proteinuria.
The diagnosis of MCNS at that time was based on minor glomerular
abnormality by the light microscopy examination. She had then
been treated with corticosteroid, after which her proteinuria
had promptly disappeared. Her corticosteroid had then been gradually
withdrawn, and no relapse had occurred for 6 years.
At her first visit with us, she had a fever and sclerodactylia as well as swollen hands and Raynaud's phenomenon, and laboratory examinations showed a high titer of anti-nuclear and anti-RNP antibodies, resulting in a diagnosis of mixed connective tissue disease (MCTD). She also had a massive proteinuria (3.2 g/day), and a kidney re-biopsy was performed (Figure 1).
She was treated again with corticosteroid (prednisolone 20 mg daily) and her proteinuria had completely disappeared within a week.
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Discussion
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The intramembranous electron dense deposits had reportedly been
revealed as secondary membranous lesions due to the collagen
disease [
1], and the histological findings in this case were
consistent with renal lesions of MCTD. However, her proteinuria
responded immediately to corticosteroid despite the presence
of glomerular basement membrane (GBM) lesions, and her clinical
course was reasonable for MCNS. As mechanisms of proteinuria
in membranous nephropathy, C5b-9 formation on the membrane of
podocytes leads to various intracellular events [
2]. These result
in degradation of GBM and redistribution of proteins that compose
the slit diaphragm, leading to development of proteinuria. In
this case, the proteinuria did probably not depend on membranous
lesions because of a rapid disappearance of urinary protein,
and it was suggested that the changing structures in GBM do
not necessarily lead to proteinuria.
Conflicts of interest statement. None declared.
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References
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- Sato H, Saito T, Yoshinaga K. Intramembranous fine deposit disease associated with collagen disorders: a new morphological entity. Virchows Arch A Pathol Anat Histopathol (1992) 420:447–451.[CrossRef][Web of Science][Medline]
- Nangaku M, Shankland SJ, Couser WG. Cellular response to injury in membranous nephropathy. J Am Soc Nephrol (2005) 16:1195–1204.[Abstract/Free Full Text]
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