Focal Segmental Glomerulosclerosis with Sjogren Syndrome: A Case Report

Year 2021, , 107 - 109, 07.03.2021

Mehmet Sezen , Abdülmecit Yıldız , Kamil Dilek , Mustafa Güllülü , Mahmut Yavuz , Ayşegül Oruç , Mehmet Fethullah Aydın , Alparslan Ersoy

https://doi.org/10.46310/tjim.876204

Abstract

Sjogren's syndrome (SjS) is a chronic, slow-progressing, autoimmune and lymphoproliferative disease. The main symptoms of the syndrome are xerostomia and keratoconjuctivitis sicca as a result of chronic inflammatory infiltration of the salivary and lacrimal glands. Focal Segmental Glomerulosclerosis complicating primary Sjögren's syndrome is extremely rare. We report on a 39-year-old woman with 5 years history of sjogren syndrome who was found to have nephrotic syndrome. Histopathological findings in kidney biopsy compatible with focal segmental glomerulosclerosis. Steroid therapy was initiated (oral prednisolone at a dose of 15 mg/day) and Cyclosporine 2 * 100mg/d. FSGS, which is associated with sjögren's syndrome, was considered suitable for the presentation because of its rare occurrence

Keywords

sjogren syndrome, focal segmental glomerulosclerosis, nephrotic syndrome

References

• 1. Srinivasan S, Slomovic AR. Sjögren syndrome. Compr Ophthalmol Update. 2007 Jul-Aug;8(4):205-12. PMID: 17999834.
• 2. Jonsson R, Vogelsang P, Volchenkov R, Espinosa A, Wahren-Herlenius M, Appel S. The complexity of Sjögren's syndrome: novel aspects on pathogenesis. Immunol Lett. 2011 Dec 30;141(1):1-9. doi: 10.1016/j.imlet.2011.06.007. Epub 2011 Jul 12. PMID: 21777618.
• 3. Yang HX, Wang J, Wen YB, et al. Renal involvement in primary Sjögren’s syndrome: a retroprospective study of 103 biopsy-proven cases from a single center in China. Int J Reumat Dis. 2017;21:222-8.
• 4. Kidder D, Rutherford E, Kipgen D, Fleming S, Geddes C, Stewart GA. Kidney biopsy findings in primary Sjögren syndrome. Nephrol Dial Transplant. 2015;30:1363-9.
• 5. D’Agati V. D., Fogo A. B., Bruijn J. A., Jennette J. C. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. American Journal of Kidney Diseases. 2004;43(2):368–382. doi: 10.1053/j.ajkd.2003.10.024.
• 6. A. V. Goules, I. P. Tatouli, H. M. Moutsopoulos, and A. G. Tzioufas, “Clinically significant renal involvement in primary Sjögren’s syndrome: clinical presentation and outcome,” Arthritis & Rheumatism, vol. 65, no. 11, pp. 2945–2953, 2013.
• 7. S. Maripuri, J. P. Grande, T. G. Osborn et al., “Renal involvement in primary Sjögren’s syndrome: a clinicopathologic study,” Clinical Journal of the American Society of Nephrology, vol. 4, no. 9, pp. 1423–1431, 2009.
• 8. 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, vol. 2019, Article ID 1749795, 6 pages, 2019.
• 9. Ren H., Wang W. M., Chen X. N., et al. Renal involvement and followup of 130 patients with primary Sjögren’s syndrome. Journal of Rheumatology. 2008;35(2):278–284.
• 10. Jasiek M., Karras A., Le Guern V., et al. A multicentre study of 95 biopsy-proven cases of renal disease in primary Sjögren’s syndrome. Rheumatology. 2017;56(3):362–370