Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome

Valerie M. Harris, Rohan Sharma, Joshua Cavett, Biji T. Kurien, Ke Liu, Kristi A. Koelsch, Astrid Rasmussen, Lida Radfar, David Lewis, Donald U. Stone, C. Erick Kaufman, Shibo Li, Barbara Segal, Daniel J. Wallace, Michael H. Weisman, Swamy Venuturupalli, Jennifer A. Kelly, Marta E. Alarcon-Riquelme, Bernardo Pons-Estel, Roland Jonsson & 28 others Xianglan Lu, Jacques Eric Gottenberg, Juan Manuel Anaya, Deborah S. Cunninghame-Graham, Andrew J W Huang, Michael T. Brennan, Pamela Hughes, Ilias Alevizos, Corinne Miceli-Richard, Edward C. Keystone, Vivian P. Bykerk, Gideon Hirschfield, Gang Xie, Wan Fai Ng, Gunnel Nordmark, Sara Magnusson Bucher, Per Eriksson, Roald Omdal, Nelson L. Rhodus, Maureen Rischmueller, Michael Rohrer, Marie Wahren-Herlenius, Torsten Witte, Xavier Mariette, Christopher J. Lessard, John B. Harley, Kathy L. Sivils, R. Hal Scofield

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Primary Sjögren's syndrome (pSS) has a strong female bias. We evaluated an X chromosome dose effect by analyzing 47,XXY (Klinefelter's syndrome, 1 in 500 live male births) among subjects with pSS. 47,XXY was determined by examination of fluorescence intensity of single nucleotide polymorphisms from the X and Y chromosomes. Among 136 pSS men there were 4 with 47,XXY. This was significantly different from healthy controls (1 of 1254 had 47,XXY, p = 0.0012 by Fisher's exact test) as well men with rheumatoid arthritis (0 of 363 with 47,XXY), but not different compared to men with systemic lupus erythematosus (SLE) (4 of 136 versus 8 of 306, Fisher's exact test p = NS). These results are consistent with the hypothesis that the number of X chromosomes is critical for the female bias of pSS, a property that may be shared with SLE but not RA.

Original languageEnglish (US)
Pages (from-to)25-29
Number of pages5
JournalClinical Immunology
Volume168
DOIs
StatePublished - Jul 1 2016
Externally publishedYes

Fingerprint

Klinefelter Syndrome
X Chromosome
Systemic Lupus Erythematosus
Y Chromosome
Live Birth
Single Nucleotide Polymorphism
Rheumatoid Arthritis
Fluorescence

Keywords

  • Klinefelter's syndrome
  • Sex bias
  • Sjögren's syndrome
  • X chromosome

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Medicine(all)

Cite this

Harris, V. M., Sharma, R., Cavett, J., Kurien, B. T., Liu, K., Koelsch, K. A., ... Scofield, R. H. (2016). Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome. Clinical Immunology, 168, 25-29. https://doi.org/10.1016/j.clim.2016.04.002

Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome. / Harris, Valerie M.; Sharma, Rohan; Cavett, Joshua; Kurien, Biji T.; Liu, Ke; Koelsch, Kristi A.; Rasmussen, Astrid; Radfar, Lida; Lewis, David; Stone, Donald U.; Kaufman, C. Erick; Li, Shibo; Segal, Barbara; Wallace, Daniel J.; Weisman, Michael H.; Venuturupalli, Swamy; Kelly, Jennifer A.; Alarcon-Riquelme, Marta E.; Pons-Estel, Bernardo; Jonsson, Roland; Lu, Xianglan; Gottenberg, Jacques Eric; Anaya, Juan Manuel; Cunninghame-Graham, Deborah S.; Huang, Andrew J W; Brennan, Michael T.; Hughes, Pamela; Alevizos, Ilias; Miceli-Richard, Corinne; Keystone, Edward C.; Bykerk, Vivian P.; Hirschfield, Gideon; Xie, Gang; Ng, Wan Fai; Nordmark, Gunnel; Bucher, Sara Magnusson; Eriksson, Per; Omdal, Roald; Rhodus, Nelson L.; Rischmueller, Maureen; Rohrer, Michael; Wahren-Herlenius, Marie; Witte, Torsten; Mariette, Xavier; Lessard, Christopher J.; Harley, John B.; Sivils, Kathy L.; Scofield, R. Hal.

In: Clinical Immunology, Vol. 168, 01.07.2016, p. 25-29.

Research output: Contribution to journalArticle

Harris, VM, Sharma, R, Cavett, J, Kurien, BT, Liu, K, Koelsch, KA, Rasmussen, A, Radfar, L, Lewis, D, Stone, DU, Kaufman, CE, Li, S, Segal, B, Wallace, DJ, Weisman, MH, Venuturupalli, S, Kelly, JA, Alarcon-Riquelme, ME, Pons-Estel, B, Jonsson, R, Lu, X, Gottenberg, JE, Anaya, JM, Cunninghame-Graham, DS, Huang, AJW, Brennan, MT, Hughes, P, Alevizos, I, Miceli-Richard, C, Keystone, EC, Bykerk, VP, Hirschfield, G, Xie, G, Ng, WF, Nordmark, G, Bucher, SM, Eriksson, P, Omdal, R, Rhodus, NL, Rischmueller, M, Rohrer, M, Wahren-Herlenius, M, Witte, T, Mariette, X, Lessard, CJ, Harley, JB, Sivils, KL & Scofield, RH 2016, 'Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome', Clinical Immunology, vol. 168, pp. 25-29. https://doi.org/10.1016/j.clim.2016.04.002
Harris, Valerie M. ; Sharma, Rohan ; Cavett, Joshua ; Kurien, Biji T. ; Liu, Ke ; Koelsch, Kristi A. ; Rasmussen, Astrid ; Radfar, Lida ; Lewis, David ; Stone, Donald U. ; Kaufman, C. Erick ; Li, Shibo ; Segal, Barbara ; Wallace, Daniel J. ; Weisman, Michael H. ; Venuturupalli, Swamy ; Kelly, Jennifer A. ; Alarcon-Riquelme, Marta E. ; Pons-Estel, Bernardo ; Jonsson, Roland ; Lu, Xianglan ; Gottenberg, Jacques Eric ; Anaya, Juan Manuel ; Cunninghame-Graham, Deborah S. ; Huang, Andrew J W ; Brennan, Michael T. ; Hughes, Pamela ; Alevizos, Ilias ; Miceli-Richard, Corinne ; Keystone, Edward C. ; Bykerk, Vivian P. ; Hirschfield, Gideon ; Xie, Gang ; Ng, Wan Fai ; Nordmark, Gunnel ; Bucher, Sara Magnusson ; Eriksson, Per ; Omdal, Roald ; Rhodus, Nelson L. ; Rischmueller, Maureen ; Rohrer, Michael ; Wahren-Herlenius, Marie ; Witte, Torsten ; Mariette, Xavier ; Lessard, Christopher J. ; Harley, John B. ; Sivils, Kathy L. ; Scofield, R. Hal. / Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome. In: Clinical Immunology. 2016 ; Vol. 168. pp. 25-29.
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abstract = "Primary Sj{\"o}gren's syndrome (pSS) has a strong female bias. We evaluated an X chromosome dose effect by analyzing 47,XXY (Klinefelter's syndrome, 1 in 500 live male births) among subjects with pSS. 47,XXY was determined by examination of fluorescence intensity of single nucleotide polymorphisms from the X and Y chromosomes. Among 136 pSS men there were 4 with 47,XXY. This was significantly different from healthy controls (1 of 1254 had 47,XXY, p = 0.0012 by Fisher's exact test) as well men with rheumatoid arthritis (0 of 363 with 47,XXY), but not different compared to men with systemic lupus erythematosus (SLE) (4 of 136 versus 8 of 306, Fisher's exact test p = NS). These results are consistent with the hypothesis that the number of X chromosomes is critical for the female bias of pSS, a property that may be shared with SLE but not RA.",
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AU - Harris, Valerie M.

AU - Sharma, Rohan

AU - Cavett, Joshua

AU - Kurien, Biji T.

AU - Liu, Ke

AU - Koelsch, Kristi A.

AU - Rasmussen, Astrid

AU - Radfar, Lida

AU - Lewis, David

AU - Stone, Donald U.

AU - Kaufman, C. Erick

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AU - Wallace, Daniel J.

AU - Weisman, Michael H.

AU - Venuturupalli, Swamy

AU - Kelly, Jennifer A.

AU - Alarcon-Riquelme, Marta E.

AU - Pons-Estel, Bernardo

AU - Jonsson, Roland

AU - Lu, Xianglan

AU - Gottenberg, Jacques Eric

AU - Anaya, Juan Manuel

AU - Cunninghame-Graham, Deborah S.

AU - Huang, Andrew J W

AU - Brennan, Michael T.

AU - Hughes, Pamela

AU - Alevizos, Ilias

AU - Miceli-Richard, Corinne

AU - Keystone, Edward C.

AU - Bykerk, Vivian P.

AU - Hirschfield, Gideon

AU - Xie, Gang

AU - Ng, Wan Fai

AU - Nordmark, Gunnel

AU - Bucher, Sara Magnusson

AU - Eriksson, Per

AU - Omdal, Roald

AU - Rhodus, Nelson L.

AU - Rischmueller, Maureen

AU - Rohrer, Michael

AU - Wahren-Herlenius, Marie

AU - Witte, Torsten

AU - Mariette, Xavier

AU - Lessard, Christopher J.

AU - Harley, John B.

AU - Sivils, Kathy L.

AU - Scofield, R. Hal

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