Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review
Background. Renal tubular acidosis and tubulointerstitial nephritis constitute the primary renal complications associated with Sjögren’s syndrome (SjS), and glomerulonephritis and nephrotic syndrome are rare. Case Presentation. A 79-year-old Japanese woman presented with bilateral leg edema and weig...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2019/1749795 |
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| author | Shigekazu Kurihara Makoto Harada Tohru Ichikawa Takashi Ehara Mamoru Kobayashi |
| author_facet | Shigekazu Kurihara Makoto Harada Tohru Ichikawa Takashi Ehara Mamoru Kobayashi |
| author_sort | Shigekazu Kurihara |
| collection | DOAJ |
| description | Background. Renal tubular acidosis and tubulointerstitial nephritis constitute the primary renal complications associated with Sjögren’s syndrome (SjS), and glomerulonephritis and nephrotic syndrome are rare. Case Presentation. 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. Conclusion. 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. |
| format | Article |
| id | doaj-art-07694c4f7e2c4d2ba1942ec7e2f2c5f5 |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-07694c4f7e2c4d2ba1942ec7e2f2c5f52025-08-20T03:55:28ZengWileyCase Reports in Rheumatology2090-68892090-68972019-01-01201910.1155/2019/17497951749795Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature ReviewShigekazu Kurihara0Makoto Harada1Tohru Ichikawa2Takashi Ehara3Mamoru Kobayashi4Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, JapanDepartment of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, JapanDepartment of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, JapanGraduate School of Health Sciences, Matsumoto University, 2095-1 Niimura, Matsumoto 390-1241, JapanDepartment of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano 380-8582, JapanBackground. Renal tubular acidosis and tubulointerstitial nephritis constitute the primary renal complications associated with Sjögren’s syndrome (SjS), and glomerulonephritis and nephrotic syndrome are rare. Case Presentation. 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. Conclusion. 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.http://dx.doi.org/10.1155/2019/1749795 |
| spellingShingle | Shigekazu Kurihara Makoto Harada Tohru Ichikawa Takashi Ehara Mamoru Kobayashi Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review Case Reports in Rheumatology |
| title | Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review |
| title_full | Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review |
| title_fullStr | Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review |
| title_full_unstemmed | Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review |
| title_short | Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review |
| title_sort | nephrotic syndrome due to focal segmental glomerulosclerosis complicating sjogren s syndrome a case report and literature review |
| url | http://dx.doi.org/10.1155/2019/1749795 |
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