Abstract
A 56-year-old male had periodic fever for 5 years and suffered from auditory hallucination and hearing impairment for 3 years. Xerostomia, xerophthalmia, elevated anti-SSA/Ro tilter, positive Schirmer’s test, and lymphocyte infiltrate of mucoserous gland in lip biopsy of this case confirmed the diagnosis of primary Sjogren’s syndrome (pSS). We review literature for fever and neuropsychiatric involvement in pSS case series. Though fever is present in 6–41 % pSS cases, periodic fever has not been reported. Auditory hallucination was rare in cases with pSS. The literature review alerts clinicians that fever and neurological manifestations were not uncommon in pSS cases.
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Mitoma J, Miyazaki T, Sutton-Smith M et al (2009) The N-glycolyl form of mouse sialyl Lewis X is recognized by selectins but not by HECA-452 and FH6 antibodies that were raised against human cells. Glycoconj J 26(5):511–523
Lee D, Schultz JB, Knauf PA, King MR (2007) Mechanical shedding of L-selectin from the neutrophil surface during rolling on sialyl Lewis x under flow. J Biol Chem 282(7):4812–4820
Hanashima S, Castagner B, Esposito D, Nokami T, Seeberger PH (2007) Synthesis of a sialic acid alpha(2-3) galactose building block and its use in a linear synthesis of sialyl Lewis X. Org Lett 9(9):1777–1779
Matsuda M, Kishida D, Tsuchiya-Suzuki A et al (2010) Periodic peritonitis due to familial Mediterranean fever in a patient with systemic lupus erythematosus. Intern Med 49(20):2259–2262
Csavas M, Majer G, Herczeg M et al (2011) Investigation of glycosylating properties of 1-deoxy-1-ethoxysulfonyl-hept-2-ulopyranosyl derivatives. Synthesis of a new sulfonic acid mimetic of the sialyl Lewis X tetrasaccharide. Carbohydr Res 346(12):1527–1533
Wei J, Cui L, Liu F et al (2010) E-selectin and Sialyl Lewis X expression is associated with lymph node metastasis of invasive micropapillary carcinoma of the breast. Int J Surg Pathol 18(3):193–200
Soliotis FC, Mavragani CP, Moutsopoulos HM (2004) Central nervous system involvement in Sjogren’s syndrome. Ann Rheum Dis 63(6):616–620
Kawar ZS, Johnson TK, Natunen S, Lowe JB, Cummings RD (2008) PSGL-1 from the murine leukocytic cell line WEHI-3 is enriched for core 2-based O-glycans with sialyl Lewis x antigen. Glycobiology 18(6):441–446
Cox PD, Hales RE (1999) CNS Sjogren’s syndrome: an underrecognized and underappreciated neuropsychiatric disorder. J Neuropsychiatry Clin Neurosci 11(2):241–247
Ampelas JF, Wattiaux MJ, Van Amerongen AP (2001) Psychiatric manifestations of lupus erythematosus systemic and Sjogren’s syndrome. Encephale 27(6):588–599
Caselli RJ, Scheithauer BW, Bowles CA et al (1991) The treatable dementia of Sjogren’s syndrome. Ann Neurol 30(1):98–101
Lin DF, Yan SM, Zhao Y et al (2010) Clinical and prognostic characteristics of 573 cases of primary Sjogren’s syndrome. Chin Med J (Engl) 123(22):3252–3257
Chen GY, Osada H, Santamaria-Babi LF, Kannagi R (2006) Interaction of GATA-3/T-bet transcription factors regulates expression of sialyl Lewis X homing receptors on Th1/Th2 lymphocytes. Proc Natl Acad Sci U S A 103(45):16894–16899
Ramos-Casals M, Brito-Zeron P, Yague J et al (2005) Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren’s syndrome. Rheumatology (Oxford) 44(1):89–94
Ishibashi Y, Inouye Y, Okano T, Taniguchi A (2005) Regulation of sialyl-Lewis x epitope expression by TNF-alpha and EGF in an airway carcinoma cell line. Glycoconj J 22(1–2):53–62
Garcia-Carrasco M, Ramos-Casals M, Rosas J et al (2002) Primary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 81(4):270–280
Higai K, Miyazaki N, Azuma Y, Matsumoto K (2006) Interleukin-1beta induces sialyl Lewis X on hepatocellular carcinoma HuH-7 cells via enhanced expression of ST3Gal IV and FUT VI gene. FEBS Lett 580(26):6069–6075
Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM (2000) Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum 29(5):296–304
Davidson BK, Kelly CA, Griffiths ID (1999) Primary Sjogren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology (Oxford) 38(3):245–253
Tajima Y, Mito Y, Owada Y, Tsukishima E, Moriwaka F, Tashiro K (1997) Neurological manifestations of primary Sjogren’s syndrome in Japanese patients. Intern Med 36(10):690–693
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Wang, HC., Chang, K., Lin, CY. et al. Periodic fever as the manifestation of primary Sjogren’s syndrome: a case report and literature review. Clin Rheumatol 31, 1517–1519 (2012). https://doi.org/10.1007/s10067-012-2039-8
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DOI: https://doi.org/10.1007/s10067-012-2039-8