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; 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; David M Lewis

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, 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 LuJacques-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, David M Lewis

Immunology and Immunotherapy

Research output: Contribution to journal › Article › peer-review

36 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 language	English
Pages (from-to)	25-9
Number of pages	5
Journal	Clinical Immunology
Volume	168
Early online date	22 Apr 2016
DOIs	https://doi.org/10.1016/j.clim.2016.04.002
Publication status	Published - Jul 2016

Keywords

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

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10.1016/j.clim.2016.04.002

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Harris, V. M., Sharma, R., Cavett, J., Kurien, B. T., Liu, K., Koelsch, K. A., Rasmussen, A., Radfar, L., 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., Jonsson, R., ... Lewis, D. M. (2016). Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome. Clinical Immunology, 168, 25-9. Advance online publication. https://doi.org/10.1016/j.clim.2016.04.002

@article{82820a4ff5d24ef9b9936c3ab504a58c,

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

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 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 Jacques-Eric Gottenberg and Juan-Manuel Anaya 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 Wan-Fai Ng 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} and Lewis, {David M}",

note = "Published by Elsevier Inc.",

year = "2016",

month = jul,

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

language = "English",

volume = "168",

pages = "25--9",

journal = "Clinical Immunology",

issn = "1521-6616",

publisher = "Elsevier",

}

Harris, VM, Sharma, R, Cavett, J, Kurien, BT, Liu, K, Koelsch, KA, Rasmussen, A, Radfar, L, 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, J-E, Anaya, J-M, Cunninghame-Graham, DS, Huang, AJW, Brennan, MT, Hughes, P, Alevizos, I, Miceli-Richard, C, Keystone, EC, Bykerk, VP, Hirschfield, G, Xie, G, Ng, W-F, 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 & Lewis, DM 2016, 'Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome', Clinical Immunology, vol. 168, pp. 25-9. https://doi.org/10.1016/j.clim.2016.04.002

TY - JOUR

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 - 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

AU - Lewis, David M

N1 - Published by Elsevier Inc.

PY - 2016/7

Y1 - 2016/7

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.

KW - Sjögren's syndrome

KW - X chromosome

KW - Klinefelter's syndrome

KW - Sex bias

U2 - 10.1016/j.clim.2016.04.002

DO - 10.1016/j.clim.2016.04.002

M3 - Article

C2 - 27109640

SN - 1521-6616

VL - 168

SP - 25

EP - 29

JO - Clinical Immunology

JF - Clinical Immunology

ER -

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

Abstract

Keywords

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