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; 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

doi:10.1016/j.clim.2016.04.002

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 JonssonXianglan 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

Diagnostic and Biological Sciences

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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 language	English (US)
Pages (from-to)	25-29
Number of pages	5
Journal	Clinical Immunology
Volume	168
DOIs	https://doi.org/10.1016/j.clim.2016.04.002
State	Published - Jul 1 2016

Bibliographical note

Funding Information:
This work has been supported in part by NIH grants AR053483 , AI082714 , AR053734 and GM104938 as well as by a Merit Review Award from the US Department of Veterans Affairs.

Publisher Copyright:
© 2016.

Keywords

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

Publisher link

10.1016/j.clim.2016.04.002

http://europepmc.org/articles/pmc4940221

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Harris, V. M., Sharma, R., Cavett, J., Kurien, B. T., Liu, K., Koelsch, K. A., Rasmussen, A., Radfar, L., Lewis, D., Stone, D. U., Kaufman, C. E., Li, S., Segal, B., Wallace, D. J., Weisman, M. H., Venuturupalli, S., Kelly, J. A., Alarcon-Riquelme, M. E., Pons-Estel, B., ... 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

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

@article{6b8f9b82e19d405ea79d94e3fbd8cd95,

title = "Klinefelter's syndrome (47,XXY) is in excess among men with Sj{\"o}gren's syndrome",

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.",

keywords = "Klinefelter's syndrome, Sex bias, Sj{\"o}gren's syndrome, X chromosome",

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

note = "Funding Information: This work has been supported in part by NIH grants AR053483 , AI082714 , AR053734 and GM104938 as well as by a Merit Review Award from the US Department of Veterans Affairs. Publisher Copyright: {\textcopyright} 2016.",

year = "2016",

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doi = "10.1016/j.clim.2016.04.002",

language = "English (US)",

volume = "168",

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T1 - Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome

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

AU - Li, Shibo

AU - Segal, Barbara

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

N1 - Funding Information: This work has been supported in part by NIH grants AR053483 , AI082714 , AR053734 and GM104938 as well as by a Merit Review Award from the US Department of Veterans Affairs. Publisher Copyright: © 2016.

PY - 2016/7/1

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N2 - 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.

AB - 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.

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KW - Sjögren's syndrome

KW - X chromosome

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ER -

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

Abstract

Bibliographical note

Keywords

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