Sj？gren’s syndrome (SS) is a chronic autoimmune disease that occurs primarily in women, with a female-to-male ratio of 9:1. We report a case of chronic kidney disease (CKD) associated with SS in a male patient, diagnosed according to the 2016 ACR/EULAR criteria, with positive SSA/RO antibodies and a Schirmer test result of < 5 mm/5 min. Renal biopsy revealed 18 glomeruli. Pathological findings included focal and segmental endocapillary proliferation (involving 3 glomeruli), a nonspecific lymphocytic tubular infiltrate (sometimes nodular) and fibroproliferative endarteritis. Immunofluorescence revealed deposits of IgA, IgG, C3, kappa, and lambda chains. These features were consistent with membranoproliferative glomerulonephritis (MPGN). A treatment regimen consisting of prednisone and mycophenolate mofetil (MMF) led to an improvement in glomerular filtration rate (GFR). This case highlights the possibility of severe glomerular involvement such as MPGN, in the course of primary SS, even in men.
References
[1]
Fox, P.C. (2007) Autoimmune Diseases and Sj？gren’s Syndrome: An Autoimmune Exocrinopathy. AnnalsoftheNewYorkAcademyofSciences, 1098, 15-21. https://doi.org/10.1196/annals.1384.003
[2]
Fran？ois, H. and Mariette, X. (2015) Renal Involvement in Primary Sj？gren Syndrome. NatureReviewsNephrology, 12, 82-93. https://doi.org/10.1038/nrneph.2015.174
[3]
Choi, J., Lee, T.W., Bae, E., Jang, H.N., An, H.J., Chang, S., et al. (2023) Iga Nephropathy with Minimal Change Disease Associated with Primary Sj？gren’s Syndrome: A Case Report. Medicine, 102, e33892. https://doi.org/10.1097/md.0000000000033892
[4]
Shiboski, C.H., Shiboski, S.C., Seror, R., Criswell, L.A., Labetoulle, M., Lietman, T.M., et al. (2016) 2016 American College of Rheumatology/European League against Rheumatism Classification Criteria for Primary Sj？gren’s Syndrome: A Consensus and Data‐Driven Methodology Involving Three International Patient Cohorts. Arthritis&Rheumatology, 69, 35-45. https://doi.org/10.1002/art.39859
[5]
Ramírez Sepúlveda, J.I., Kvarnstr？m, M., Eriksson, P., Mandl, T., Norheim, K.B., Johnsen, S.J., et al. (2017) Long-Term Follow-Up in Primary Sj？gren’s Syndrome Reveals Differences in Clinical Presentation between Female and Male Patients. BiologyofSexDifferences, 8, Article No. 25. https://doi.org/10.1186/s13293-017-0146-6
[6]
Park, Y., Lee, J., Park, S. and Kwok, S. (2020) Male Patients with Primary Sj？gren’s Syndrome: A Distinct Clinical Subgroup? InternationalJournalofRheumaticDiseases, 23, 1388-1395. https://doi.org/10.1111/1756-185x.13940
[7]
Jain, A., Srinivas, B.H., Emmanuel, D., Jain, V.K., Parameshwaran, S. and Negi, V.S. (2018) Renal Involvement in Primary Sjogren’s Syndrome: A Prospective Cohort Study. RheumatologyInternational, 38, 2251-2262. https://doi.org/10.1007/s00296-018-4118-x
[8]
Aiyegbusi, O., McGregor, L., McGeoch, L., Kipgen, D., Geddes, C.C. and Stevens, K.I. (2020) Renal Disease in Primary Sj？gren’s Syndrome. RheumatologyandTherapy, 8, 63-80. https://doi.org/10.1007/s40744-020-00264-x
[9]
Maripuri, S., Grande, J.P., Osborn, T.G., Fervenza, F.C., Matteson, E.L., Donadio, J.V., et al. (2009) Renal Involvement in Primary Sj？gren’s Syndrome: A Clinicopathologic Study. ClinicalJournaloftheAmericanSocietyofNephrology, 4, 1423-1431. https://doi.org/10.2215/cjn.00980209
[10]
Goules, A., Geetha, D., Arend, L.J. and Baer, A.N. (2019) Renal Involvement in Primary Sj？gren’s Syndrome: Natural History and Treatment Outcome. Clinical and Experimental Rheumatology, 37, S123-S132.
[11]
Kidder, D., Rutherford, E., Kipgen, D., Fleming, S., Geddes, C. and Stewart, G.A. (2015) Kidney Biopsy Findings in Primary Sj？gren Syndrome. NephrologyDialysisTransplantation, 30, 1363-1369. https://doi.org/10.1093/ndt/gfv042
