Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren's Syndrome: A Case Report and Literature Review
Identifieur interne : 000596 ( Main/Exploration ); précédent : 000595; suivant : 000597Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren's Syndrome: A Case Report and Literature Review
Auteurs : Shigekazu Kurihara [Japon] ; Makoto Harada [Japon] ; Tohru Ichikawa [Japon] ; Takashi Ehara [Japon] ; Mamoru Kobayashi [Japon]Source :
- Case Reports in Rheumatology [ 2090-6889 ] ; 2019.
Abstract
Renal tubular acidosis and tubulointerstitial nephritis constitute the primary renal complications associated with Sjögren's syndrome (SjS), and glomerulonephritis and nephrotic syndrome are rare.
A 79-year-old Japanese woman presented with bilateral leg edema and weight gain and was diagnosed with nephrotic syndrome. In addition, she reported a 5-year history of dryness of mouth and was diagnosed with SjS. Renal biopsy revealed segmental glomerulosclerosis, with some specimens showing collapse of the glomerular capillary loops, proliferation of glomerular epithelial cells, and sclerotic lesions at the tubular poles, without spike formation, double contour lesions, or any other changes of the glomerular basement membrane. Immunofluorescence staining showed no immune complex (immunoglobulin IgG, IgA, or IgM) or complement (C3) deposition in the glomerular capillary walls. Based on these findings, she was diagnosed with focal segmental glomerulosclerosis (FSGS). The administration of steroid and cyclosporine achieved complete remission of nephrotic syndrome.
Although glomerular diseases are rare, a variety of glomerular lesions including FSGS are reported in patients with SjS. Therefore, renal biopsy is warranted in patients with SjS presenting with severe urinary abnormalities.
Url:
DOI: 10.1155/2019/1749795
PubMed: 31511795
PubMed Central: 6714328
Affiliations:
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Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582</wicri:regionArea><wicri:noRegion>Nagano 380-8582</wicri:noRegion></affiliation></author><author><name sortKey="Harada, Makoto" sort="Harada, Makoto" uniqKey="Harada M" first="Makoto" last="Harada">Makoto Harada</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, Japan</nlm:aff><country xml:lang="fr">Japon</country><wicri:regionArea>Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582</wicri:regionArea><wicri:noRegion>Nagano 380-8582</wicri:noRegion></affiliation><affiliation wicri:level="1"><nlm:aff id="I2">Department of Nephrology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan</nlm:aff><country xml:lang="fr">Japon</country><wicri:regionArea>Department of Nephrology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621</wicri:regionArea><wicri:noRegion>Matsumoto 390-8621</wicri:noRegion></affiliation></author><author><name sortKey="Ichikawa, Tohru" sort="Ichikawa, Tohru" uniqKey="Ichikawa T" first="Tohru" last="Ichikawa">Tohru Ichikawa</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, Japan</nlm:aff><country xml:lang="fr">Japon</country><wicri:regionArea>Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582</wicri:regionArea><wicri:noRegion>Nagano 380-8582</wicri:noRegion></affiliation></author><author><name sortKey="Ehara, Takashi" sort="Ehara, Takashi" uniqKey="Ehara T" first="Takashi" last="Ehara">Takashi Ehara</name><affiliation wicri:level="1"><nlm:aff id="I3">Graduate School of Health Sciences, Matsumoto University, 2095-1 Niimura, Matsumoto 390-1241, Japan</nlm:aff><country xml:lang="fr">Japon</country><wicri:regionArea>Graduate School of Health Sciences, Matsumoto University, 2095-1 Niimura, Matsumoto 390-1241</wicri:regionArea><wicri:noRegion>Matsumoto 390-1241</wicri:noRegion></affiliation></author><author><name sortKey="Kobayashi, Mamoru" sort="Kobayashi, Mamoru" uniqKey="Kobayashi M" first="Mamoru" last="Kobayashi">Mamoru Kobayashi</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, Japan</nlm:aff><country xml:lang="fr">Japon</country><wicri:regionArea>Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582</wicri:regionArea><wicri:noRegion>Nagano 380-8582</wicri:noRegion></affiliation></author></analytic><series><title level="j">Case Reports in Rheumatology</title><idno type="ISSN">2090-6889</idno><idno type="eISSN">2090-6897</idno><imprint><date when="2019">2019</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec><title>Background</title><p> Renal tubular acidosis and tubulointerstitial nephritis constitute the primary renal complications associated with Sjögren's syndrome (SjS), and glomerulonephritis and nephrotic syndrome are rare. </p></sec><sec><title>Case Presentation</title><p> A 79-year-old Japanese woman presented with bilateral leg edema and weight gain and was diagnosed with nephrotic syndrome. In addition, she reported a 5-year history of dryness of mouth and was diagnosed with SjS. Renal biopsy revealed segmental glomerulosclerosis, with some specimens showing collapse of the glomerular capillary loops, proliferation of glomerular epithelial cells, and sclerotic lesions at the tubular poles, without spike formation, double contour lesions, or any other changes of the glomerular basement membrane. Immunofluorescence staining showed no immune complex (immunoglobulin IgG, IgA, or IgM) or complement (C3) deposition in the glomerular capillary walls. Based on these findings, she was diagnosed with focal segmental glomerulosclerosis (FSGS). The administration of steroid and cyclosporine achieved complete remission of nephrotic syndrome. </p></sec><sec><title>Conclusion</title><p> Although glomerular diseases are rare, a variety of glomerular lesions including FSGS are reported in patients with SjS. 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Chen</name></author></analytic></biblStruct></listBibl></div1></back></TEI><affiliations><list><country><li>Japon</li></country></list><tree><country name="Japon"><noRegion><name sortKey="Kurihara, Shigekazu" sort="Kurihara, Shigekazu" uniqKey="Kurihara S" first="Shigekazu" last="Kurihara">Shigekazu Kurihara</name></noRegion><name sortKey="Ehara, Takashi" sort="Ehara, Takashi" uniqKey="Ehara T" first="Takashi" last="Ehara">Takashi Ehara</name><name sortKey="Harada, Makoto" sort="Harada, Makoto" uniqKey="Harada M" first="Makoto" last="Harada">Makoto Harada</name><name sortKey="Harada, Makoto" sort="Harada, Makoto" uniqKey="Harada M" first="Makoto" last="Harada">Makoto Harada</name><name sortKey="Ichikawa, Tohru" sort="Ichikawa, Tohru" uniqKey="Ichikawa T" first="Tohru" last="Ichikawa">Tohru Ichikawa</name><name sortKey="Kobayashi, Mamoru" sort="Kobayashi, Mamoru" uniqKey="Kobayashi M" first="Mamoru" last="Kobayashi">Mamoru Kobayashi</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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