CRCM  Vol.2 No.3 , June 2013
Primary Sjogren’s syndrome and focal proliferative and crescentric necrotizing glomerulonephritis
ABSTRACT

Intro: Primary Sjogren’s syndrome is a chronic systemic autoimmune disease. Although ocular and oral dryness are the hallmarks of this syndrome, renal involvement when present most commonly has the histological appearance of tubointerstitial nephritis and less often glomerular involvement. We report, a unique case of renal manifestations of primary Sjogren’s syndrome with a proven histological focal proliferative and crescentric necrotizing glomeru-lonephritis. Case: A 30 years old man with Kimura disease presented with four months of subjective fevers, migrating skin erythema, dry mouth, and dry eyes. On further workup the patient was found to have a positive ANA, positive anti-SSA, and was evaluated by ophthalmology who found keratoconjunctivitis sicca. The patient was started on hydroxychloroquine in combination with prednisone. On follow up, the patient was found to be hypertensive with acute kidney injury, a creatinine of 3.65, proteinuria and hematuria. Renal ultrasound was unremarkable. Cytoplasmic ANCA was positive and kidney biopsy was performed revealing non pauci-immune focal proliferative and necrotizing glomerulonephritis with cellular crescents and mesangial dense deposits by electron microscopy. Conclusion: To our knowledge, the association between biopsy proven focal proliferative and necrotizing glomerulonephritis with primary Sjogren’s syndrome has never been previously described in the literature, nor has there been a reported case of association between Kimura disease, ANCA serology and primary Sjogren’s syndrome.


Cite this paper
Brodsky, J. , Patton, E. and Chang, Y. (2013) Primary Sjogren’s syndrome and focal proliferative and crescentric necrotizing glomerulonephritis. Case Reports in Clinical Medicine, 2, 211-214. doi: 10.4236/crcm.2013.23057.
References
[1]   Shiboski, S.C., Shiboski, C.H., Criswell, A., et al. (2012) American college of rheumatology classification criteria for Sjögren’s syndrome: A data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Research, 64, 475-487. doi:10.1002/acr.21591

[2]   Tsokos, M., Lazarou, S.A. and Moutsopoulos, H.M. (1987) Vasculitis in primary Sjogren’s syndrome. Histologic classification and clinical presentation. American Journal of Clinical Pathology, 88, 26-31.

[3]   Itzgerald, S., Hurst, N.P. and Pannall, P.R. (1987) Sjogren’s syndrome, vasculitis, and cryoglobulinaemia associated with a monoclonal IgM (kappa) paraprotein with rheumatoid factor activity. Annals of the Rheumatic Disease, 46, 485-487. doi:10.1136/ard.46.6.485

[4]   Moutsopoulos, H.M., Balow, J.E., Lawley, T.J., et al. (1978) Immune complex glomerulonephritis in sicca syndrome. American Journal of Medicine, 64, 955-960. doi:10.1016/0002-9343(78)90449-7

[5]   Maripuri, S., Grande, J.P., Osborn, T.G., et al. (2009) Renal involvement in primary Sjögren’s syndrome: A clinicopathologic study. Clinical Journal of the American Society of Nephrology, 4, 1423-1431. doi:10.2215/CJN.00980209

[6]   Goules, A., Masouridi, S., Tzioufas, A.G., et al. (2000) Clinically significant and biopsydocumented renal involvement in primary Sjögren syndrome. Medicine (Baltimore), 79, 241-249. doi:10.1097/00005792-200007000-00005

[7]   Bossini, N., Savoldi, S., Franceschini, F., et al. (2001) Clinical and morphological features of kidney involvement in primary Sjogren’s syndrome. Nephrology Dialysis Transplantation, 16, 2328-2336. doi:10.1093/ndt/16.12.2328

[8]   Goules, A., Masouridi, S., Tzioufas, A.G., et al. (2000) Clinically significant and biopsy-documented renal involvement in primary Sjogren syndrome. Medicine (Baltimore), 79, 241-249. doi:10.1097/00005792-200007000-00005

[9]   Skopouli, F.N., Dafni, U. and Ioannidis, J.P.A. (2000) Clinical evolution and morbidity and mortality of primary Sjögren’s syndrome. Seminars in Arthritis and Rheumatism, 29, 296-304. doi:10.1016/S0049-0172(00)80016-5

[10]   Bossini, N., Savoldi, S., Franceschini, F., Mombelloni, S., et al. (2001) Clinical and morphological features of kidney involvement in primary Sjögren’s syndrome. Nephrology Dialysis Transplantation, 16, 2328-2336. doi:10.1093/ndt/16.12.2328

[11]   Font, J., Ramos-Casals, M., Cervera, R., et al. (1998) Antineutrophil cytoplasmic antibodies in primary Sjögren’s syndrome: Prevalence and clinical significance. Rheumatology, 37, 1287-1291. doi:10.1093/rheumatology/37.12.1287

[12]   Matsuda, O., Makiguchi, K., Ishibashi, K., et al. (1992) Long term effects of steroid treatment on nephrotic syndrome associated with Kimura’s disease and a review of the literature. Clinical Nephrology, 37, 119-123.

[13]   Yamada, A., Mitsuhashi, K., Miyakawa, Y., et al. (1982) Membranous glomerulonephritis associated with eosinophilic lymphfolliculosis of the skin (Kimura’s disease): Report of a case and review of the literature. Clinical Nephrology, 18, 211-215.

 
 
Top