Details of Disease

General Information of Disease (ID: DISUBX7H)

Disease Name Sjogren syndrome
Synonyms
Sjogren's syndrome; primary Sjgren syndrome; syndrome, Sjogren's; Sjgren syndrome; Sjgren-Gougerot syndrome; sicca syndrome; Sjogren syndrome; primary Sjgren-Gougerot syndrome; primary Sjogren-Gougerot syndrome; xerodermosteosis
Disease Class 4A43: Sjogren syndrome
Definition
An autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjgren syndrome is also associated with rheumatic disorders such as rheumatoid arthritis or systemic lupus erythematosus. The hallmark symptoms of Sjgren syndrome are dry mouth and dry eyes. In addition, Sjogren syndrome may cause skin, nose, and vaginal dryness. It also may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain|Editor note: check for differences with Mik PMID:10845583
Disease Hierarchy
DISOAHYA: Salivary gland disorder
DIS6SVEE: Syndromic disease
DISEMG4F: Autoimmune disorder of exocrine system
DIST4Z21: Disorder of lacrimal gland
DISUBX7H: Sjogren syndrome
ICD Code
ICD-11
ICD-11: 4A43.20
ICD-10
ICD-10: M35, M35.0
ICD-9
ICD-9: 710.2
Expand ICD-9
710.2
Disease Identifiers
MONDO ID
MONDO_0010030
MESH ID
D012859
UMLS CUI
C1527336
OMIM ID
270150
MedGen ID
282890
Orphanet ID
289390
SNOMED CT ID
83901003

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 4 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Bromhexine DMEXWWV Approved Small molecular drug [1]
Cevimeline DMWSMND Approved Small molecular drug [2]
Leniolisib DMWAD93 Approved Small molecular drug [3]
Prednisolone DMQ8FR2 Approved Small molecular drug [4]
------------------------------------------------------------------------------------
This Disease is Treated as An Indication in 14 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Nipocalimab? DM0DYAX Phase 3 Monoclonal antibody [5]
LY3090106 DM0DLGO Phase 2/3 Antibody [6]
AMG 557 DME6QH1 Phase 2 NA [7]
CFZ533 DM1TWIE Phase 2 NA [7]
Dazodalibep DM7YII9 Phase 2 NA [8]
Frexalimab DMMJTYP Phase 2 Antibody [9]
LY3361237 DMHXMYU Phase 2 Antibody [10]
RG7625 DMDGPKS Phase 2 NA [7]
RSLV-132 DMP4519 Phase 2 NA [7]
VAY736 DML2CPW Phase 2 Antibody [11]
BIIB063 DMU9SL8 Phase 1 NA [7]
GSK2618960 DMIVHDN Phase 1 NA [7]
MEDI4920 DME7BMT Phase 1 NA [12]
MLN-3126 DMDB3OK Phase 1 NA [13]
------------------------------------------------------------------------------------
⏷ Show the Full List of 14 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 62 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
APOH TT2OUI9 Limited Biomarker [14]
CA1 TTHQPL7 Limited Genetic Variation [15]
CHIA TTJB1O0 Limited Altered Expression [16]
E2F1 TTASI04 Limited Biomarker [17]
TNFRSF13C TT7NJSE Limited Genetic Variation [18]
CXCL13 TT0NIZ1 Disputed Biomarker [19]
MAP3K14 TT4LIAC Disputed Biomarker [20]
CTSS TTUMQVO moderate Biomarker [21]
ACR TTAHE2N Strong Genetic Variation [22]
BLK TTNDSC3 Strong Genetic Variation [23]
BMP6 TT07RIB Strong Altered Expression [24]
CCL21 TTLZK1U Strong Altered Expression [25]
CCR7 TT2GIDQ Strong Biomarker [25]
CCR9 TTIPS8B Strong Altered Expression [26]
CD2 TTJDUNO Strong Biomarker [27]
CD4 TTN2JFW Strong Biomarker [28]
CHRM3 TTQ13Z5 Strong Biomarker [29]
CLN3 TTORF9W Strong Biomarker [30]
CNTN2 TT2Z1WB Strong Biomarker [31]
CR2 TT0HUN7 Strong Biomarker [32]
CXCL10 TTQOVYA Strong Altered Expression [26]
CXCL11 TTWG0RE Strong Biomarker [33]
CXCL9 TTWE5PB Strong Altered Expression [34]
CXCR5 TTIW59R Strong Genetic Variation [35]
CYP19A1 TTSZLWK Strong Biomarker [36]
FAS TT7LTUJ Strong Biomarker [37]
FCGR2A TTXT21W Strong Biomarker [38]
ICA1 TTMX06B Strong Biomarker [39]
IL12A TTRTK6Y Strong Biomarker [40]
IL2 TTF89GD Strong Biomarker [41]
IL21 TT9QEJ6 Strong Biomarker [42]
IL23R TT6H4QR Strong Genetic Variation [43]
IL2RA TT10Y9E Strong Altered Expression [27]
IL4 TTLGTKB Strong Biomarker [44]
IL7 TT8FRMO Strong Biomarker [45]
ITGAE TTH0Z37 Strong Biomarker [46]
ITGAL TT48WR6 Strong Genetic Variation [47]
ITPR1 TT5HWAT Strong Biomarker [48]
ITPR3 TTH1769 Strong Biomarker [49]
KCNH3 TTZ4MTQ Strong Altered Expression [50]
KLRG1 TT299E6 Strong Genetic Variation [51]
LTBR TTFO0PM Strong Biomarker [52]
LY9 TTZCL1U Strong Biomarker [53]
MSMB TTYH1ZK Strong Biomarker [54]
MUC5AC TTEL90S Strong Altered Expression [55]
MYCBP TTVW4XU Strong Altered Expression [56]
NCF1 TTZ4JC3 Strong Biomarker [57]
NFKBIA TTSHAEB Strong Biomarker [58]
OXTR TTSCIUP Strong Biomarker [59]
PRG4 TTSKF4V Strong Biomarker [60]
PSMB9 TTOUSTQ Strong Altered Expression [61]
SLC26A9 TTKRVU0 Strong Biomarker [25]
SPTBN1 TTS9BDA Strong Biomarker [62]
SSRP1 TTETDKQ Strong Biomarker [63]
ST14 TTPRO7W Strong Genetic Variation [64]
THBS1 TTKI0H1 Strong Biomarker [65]
TNFSF13 TTOI1RM Strong Biomarker [66]
TNFSF13B TTWMIDN Strong Biomarker [67]
TRAF6 TTCDR6M Strong Biomarker [68]
TRIM27 TTTO3QN Strong Biomarker [69]
MALT1 TTCI81G Definitive Genetic Variation [70]
VIPR2 TT4O5P0 Definitive Altered Expression [71]
------------------------------------------------------------------------------------
⏷ Show the Full List of 62 DTT(s)
This Disease Is Related to 2 DTP Molecule(s)
Gene Name DTP ID Evidence Level Mode of Inheritance REF
ABCA7 DTDVSJA Disputed Biomarker [72]
SLC17A1 DT8ARWJ Strong Biomarker [69]
------------------------------------------------------------------------------------
This Disease Is Related to 4 DME Molecule(s)
Gene Name DME ID Evidence Level Mode of Inheritance REF
CHST3 DEQIZP2 Strong Biomarker [73]
NAT10 DEZV4AP Strong Biomarker [74]
TPMT DEFQ8VO Strong Biomarker [75]
UGT2B28 DEB7I4M Strong Genetic Variation [76]
------------------------------------------------------------------------------------
This Disease Is Related to 112 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
FAM167A OT9JF3JQ Limited Genetic Variation [23]
HLA-DPB1 OTW8JHU2 Limited Genetic Variation [51]
PUF60 OTG90DYF Limited Biomarker [77]
RELN OTLKMW1O Limited Genetic Variation [76]
SPRR1B OTXNW3P6 Limited Altered Expression [78]
SPRR2A OT62ZU6B Limited Altered Expression [79]
STAT4 OTAK3VFR Limited Genetic Variation [51]
TREX1 OTQG7K12 Limited Genetic Variation [80]
ARHGAP45 OTL86FEQ Disputed Genetic Variation [72]
ATN1 OTNZFLKY moderate Biomarker [81]
DNAJC10 OTD2EG0R moderate Biomarker [82]
NUDT10 OT61XMYC moderate Genetic Variation [83]
AIRE OTA7G1Y1 Strong Biomarker [84]
AMY1A OT6G4B8O Strong Altered Expression [56]
AQP4 OTA9MYD5 Strong Biomarker [85]
AQP5 OT77GBY8 Strong Altered Expression [86]
ASCL2 OT3D62WB Strong Altered Expression [87]
ATRNL1 OTY5JUX2 Strong Biomarker [74]
BTG3 OT9ANHVT Strong Genetic Variation [88]
C4A OTXMOYXU Strong Genetic Variation [89]
CCL18 OT7JYSK9 Strong Biomarker [90]
CCL25 OTLWJ8CJ Strong Biomarker [26]
CD8A OTDWQJXK Strong Biomarker [46]
CENPB OT3N73QR Strong Biomarker [29]
CHP1 OTHTXN1A Strong Biomarker [91]
CLDN5 OTUX60YO Strong Altered Expression [50]
CNN3 OTJTAXAP Strong Biomarker [92]
CRISP3 OTBSWMPL Strong Biomarker [93]
CUL9 OTPUCLZT Strong Altered Expression [94]
DGKQ OT8OEPYT Strong Biomarker [38]
DHX40 OTOL02QN Strong Biomarker [95]
DLAT OT9LBJVN Strong Biomarker [96]
DSTN OTMXO4YB Strong Altered Expression [97]
EDA OTAKS5WS Strong Biomarker [98]
ERV3-1 OT4CW64T Strong Altered Expression [99]
FUCA1 OTW71IK4 Strong Biomarker [100]
FUCA2 OTY5NVMJ Strong Biomarker [100]
GNL3 OTILGYO4 Strong Altered Expression [101]
GOLGA1 OTBBPBG0 Strong Biomarker [102]
GOLGA4 OTCMEHNJ Strong Genetic Variation [103]
GRAP OTO1P7YX Strong Biomarker [104]
GTF2I OTUYL1TQ Strong Genetic Variation [105]
GZMA OT43R33L Strong Altered Expression [106]
HEYL OTN7BH79 Strong Biomarker [107]
HLA-DRA OT7KZMP2 Strong Genetic Variation [38]
HLA-DRB3 OT5PM9N7 Strong Biomarker [108]
HMCN1 OTLZAS0P Strong Altered Expression [109]
HOXC6 OTBCRAZV Strong Biomarker [110]
ID3 OTUULW5Z Strong Biomarker [42]
IFI16 OT4SPU0U Strong Biomarker [111]
IFNLR1 OTWWWK45 Strong Biomarker [112]
IKBKE OT5VYOSM Strong Biomarker [113]
IL12RB1 OTM1IJO2 Strong Genetic Variation [114]
IL17RB OT0KDNSF Strong Altered Expression [68]
ILF2 OTWWVM9X Strong Biomarker [115]
ILF3 OTKMZ5K5 Strong Biomarker [115]
IRAK1BP1 OTRSC1CD Strong Biomarker [38]
IRF2 OTAZRUW3 Strong Altered Expression [116]
IRF5 OT8SIIAP Strong Genetic Variation [51]
ITSN2 OT8S0OO8 Strong Biomarker [38]
KRT6B OTBXJYHY Strong Biomarker [34]
LAMTOR2 OTHEDISB Strong Altered Expression [117]
LAPTM5 OT2XI2JG Strong Altered Expression [118]
LCN1 OT8BWXTV Strong Biomarker [119]
LILRA3 OTBNQCOS Strong Biomarker [120]
MSH5 OTDARQT3 Strong Genetic Variation [76]
MUC19 OTM06UGV Strong Altered Expression [121]
MUC5B OTPW6K5C Strong Biomarker [122]
MYDGF OT9HRPL6 Strong Genetic Variation [123]
NAPB OTCUENKD Strong Genetic Variation [76]
NCR3 OT20M764 Strong Biomarker [124]
NFKBIL1 OTYFTPS0 Strong Genetic Variation [125]
NRSN1 OT1KKXC8 Strong Biomarker [91]
NT5C1A OTS7UF36 Strong Biomarker [126]
NUDT15 OTX8SZOT Strong Genetic Variation [75]
OAS1 OT8ZLOCY Strong Genetic Variation [127]
PDLIM3 OTVXQC81 Strong Biomarker [74]
PHIP OTZY806Z Strong Biomarker [38]
PPBP OT1FHGQS Strong Altered Expression [128]
PRAMEF13 OTVJTEXT Strong Genetic Variation [76]
PRDM1 OTQLSVBS Strong Biomarker [38]
PSPH OTV1PVAX Strong Biomarker [54]
PSPN OT54LLZJ Strong Biomarker [54]
PTTG1 OTIMYS4W Strong Biomarker [38]
RAB3D OTGKRR3C Strong Altered Expression [129]
RAB4A OT7BL9WW Strong Altered Expression [130]
RBBP4 OTG3BT3M Strong Altered Expression [131]
RBM45 OTWTHD77 Strong Biomarker [132]
REG1A OTMHUH1D Strong Biomarker [54]
RIDA OTW4098I Strong Biomarker [54]
RNF125 OTBD1Q3X Strong Genetic Variation [133]
RNPC3 OTW5MKC1 Strong Biomarker [134]
RO60 OTLGM5A8 Strong Biomarker [135]
RPP14 OT4OYFSK Strong Altered Expression [117]
SEMA7A OT0ZJK64 Strong Altered Expression [136]
SF3B6 OTPRKS6S Strong Altered Expression [117]
SHISA9 OTZI2V0J Strong Genetic Variation [51]
SPTAN1 OT6VY3A3 Strong Biomarker [137]
SSB OTCCTPBR Strong Biomarker [138]
STXBP3 OTTTYMAQ Strong Biomarker [54]
TAP2 OTWSYFI7 Strong Biomarker [139]
TARBP1 OT14Z6RJ Strong Genetic Variation [76]
TMED2 OTBLO7RW Strong Biomarker [91]
TMEM132D OTV6I4Z0 Strong Genetic Variation [140]
TNPO3 OTOT3HH0 Strong Genetic Variation [38]
TPPP OTCFMSUF Strong Biomarker [91]
TRAF3IP2 OTLLZERL Strong Genetic Variation [43]
TRAT1 OTMPUNPD Strong Biomarker [141]
TRIM22 OTJBLG5R Strong Biomarker [141]
TRIM38 OT6SP1M9 Strong Biomarker [141]
TRIM68 OTHMDMOG Strong Biomarker [142]
EDA2R OTJLAIIH Definitive Biomarker [143]
------------------------------------------------------------------------------------
⏷ Show the Full List of 112 DOT(s)

References

1 Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
2 Therapeutic effect of cevimeline on dry eye in patients with Sj gren's syndrome: a randomized, double-blind clinical study. Am J Ophthalmol. 2004 Jul;138(1):6-17.
3 ClinicalTrials.gov (NCT02775916) Safety, Pharmacokinetics, and Preliminary Efficacy Study of CDZ173 in Patients With Primary Sjogren's Syndrome. U.S. National Institutes of Health.
4 Prednisolone FDA Label
5 ClinicalTrials.gov (NCT04968912) A Randomized, Placebo-controlled, Double-blind, Multicenter Study to Assess the Efficacy and Safety of Nipocalimab in Adults With Primary Sjogren's Syndrome (pSS). U.S.National Institutes of Health.
6 ClinicalTrials.gov (NCT04563195) BAFF/IL-17 Bispecific Antibody Treatment in Subjects With Primary Sjogren's Syndrome (BAFF/IL-17). U.S. National Institutes of Health.
7 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
8 ClinicalTrials.gov (NCT04129164) A Phase 2 Randomized, Double-blind, Placebo-controlled, Proof of Concept Study to Evaluate the Efficacy and Safety of VIB4920 in Subjects With Sj?gren's Syndrome (SS). U.S.National Institutes of Health.
9 ClinicalTrials.gov (NCT04572841) A Randomized, Double-blind, Placebo-controlled, Parallel-group Study of the Safety, Tolerability, Pharmacokinetics, and Therapeutic Efficacy of SAR441344 in Adult Patients With Primary Sj?gren's Syndrome (pSjS). U.S.National Institutes of Health.
10 ClinicalTrials.gov (NCT05781451) A Phase 2, Open Label Study of Anti-BTLA Agonist Therapy in Subjects With Primary Sjogren's Syndrome. U.S.National Institutes of Health.
11 ClinicalTrials.gov (NCT02137889) Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of VAY736 in Relapsed or Refractory CLL Patients. U.S. National Institutes of Health.
12 ClinicalTrials.gov (NCT02151110) A Phase 1, Randomized, Blinded, Placebo-controlled, Single-ascending Dose Study to Evaluate the Safety and Tolerability of MEDI4920 in Healthy Adults. U.S. National Institutes of Health.
13 ClinicalTrials.gov (NCT02209506) Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of MLN3126 in Healthy Japanese and Non-Japanese Participants. U.S. National Institutes of Health.
14 Anti-beta2-glycoprotein I in Sjogren's syndrome is associated with parkinsonism.Clin Rheumatol. 2007 May;26(5):743-7. doi: 10.1007/s10067-006-0398-8. Epub 2006 Oct 21.
15 HLA-DQB1 CAR1/CAR2, TNFa IR2/IR4 and CTLA-4 polymorphisms in Tunisian patients with rheumatoid arthritis and Sjgren's syndrome.Rheumatology (Oxford). 2001 Dec;40(12):1370-4. doi: 10.1093/rheumatology/40.12.1370.
16 Acidic mammalian chitinase in dry eye conditions.Cornea. 2009 Jul;28(6):667-72. doi: 10.1097/ICO.0b013e31819bc308.
17 E2f1 mutation induces early onset of diabetes and Sjgren's syndrome in nonobese diabetic mice.J Immunol. 2004 Oct 15;173(8):4908-18. doi: 10.4049/jimmunol.173.8.4908.
18 A BAFF receptor His159Tyr mutation in Sjgren's syndrome-related lymphoproliferation.Arthritis Rheumatol. 2015 Oct;67(10):2732-41. doi: 10.1002/art.39231.
19 CXCL13 as biomarker for histological involvement in Sjgren's syndrome.Rheumatology (Oxford). 2020 Jan 1;59(1):165-170. doi: 10.1093/rheumatology/kez255.
20 Structure of thymic cysts in congenital lymph nodes-lacking mice.Anat Histol Embryol. 2008 Apr;37(2):126-30. doi: 10.1111/j.1439-0264.2007.00806.x. Epub 2007 Nov 6.
21 Longitudinal analysis of tear cathepsin S activity levels in male non-obese diabetic mice suggests its potential as an early stage biomarker of Sjgren's Syndrome.Biomarkers. 2019 Feb;24(1):91-102. doi: 10.1080/1354750X.2018.1514656. Epub 2018 Sep 12.
22 Neuro-Sjgren: Peripheral Neuropathy With Limb Weakness in Sjgren's Syndrome.Front Immunol. 2019 Jul 11;10:1600. doi: 10.3389/fimmu.2019.01600. eCollection 2019.
23 The rheumatic disease-associated FAM167A-BLK locus encodes DIORA-1, a novel disordered protein expressed highly in bronchial epithelium and alveolar macrophages.Clin Exp Immunol. 2018 Aug;193(2):167-177. doi: 10.1111/cei.13138. Epub 2018 May 16.
24 Bone Morphogenetic Protein 6 Inhibits the Immunomodulatory Property of BMMSCs via Id1 in Sjgren's Syndrome.Stem Cells Int. 2018 Aug 2;2018:9837035. doi: 10.1155/2018/9837035. eCollection 2018.
25 Laser microdissection coupled with RNA-seq reveal cell-type and disease-specific markers in the salivary gland of Sjgren's syndrome patients.Clin Exp Rheumatol. 2017 Sep-Oct;35(5):777-785. Epub 2017 Apr 18.
26 Decreased circulating CXCR3+CCR9+T helper cells are associated with elevated levels of their ligands CXCL10 and CCL25 in the salivary gland of patients with Sjgren's syndrome to facilitate their concerted migration.Scand J Immunol. 2020 Mar;91(3):e12852. doi: 10.1111/sji.12852. Epub 2019 Dec 13.
27 RORt antagonist improves Sjgren's syndrome-like sialadenitis through downregulation of CD25.Oral Dis. 2020 May;26(4):766-777. doi: 10.1111/odi.13255. Epub 2020 Mar 3.
28 Decreased CD4+CD25+bright T cells in peripheral blood of patients with primary Sjogren's syndrome.Lupus. 2008 Jan;17(1):34-9. doi: 10.1177/0961203307085248.
29 Reproducibility of fluid-phase measurements in PBS-treated sputum supernatant of healthy and stable COPD subjects.Int J Chron Obstruct Pulmon Dis. 2019 Apr 11;14:835-852. doi: 10.2147/COPD.S187661. eCollection 2019.
30 Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: further evidence that CLN3P has anti-apoptotic properties.Mol Genet Metab. 2006 Jun;88(2):178-83. doi: 10.1016/j.ymgme.2006.01.011. Epub 2006 Mar 3.
31 Detection of the tax gene of HTLV-I in labial salivary glands from patients with Sjgren's syndrome and other diseases of the oral cavity.Clin Exp Rheumatol. 2000 May-Jun;18(3):341-7.
32 Expansion of autoreactive unresponsive CD21-/low B cells in Sjgren's syndrome-associated lymphoproliferation.Arthritis Rheum. 2013 Apr;65(4):1085-96. doi: 10.1002/art.37828.
33 Disruption of CXCR3 function impedes the development of Sjgren's syndrome-like xerostomia in non-obese diabetic mice.Lab Invest. 2018 May;98(5):620-628. doi: 10.1038/s41374-017-0013-4. Epub 2018 Jan 18.
34 Up-regulated gene expression in the conjunctival epithelium of patients with Sjgren's syndrome.Exp Eye Res. 2003 Jul;77(1):17-26. doi: 10.1016/s0014-4835(03)00087-3.
35 Diminished CXCR5 expression in peripheral blood of patients with Sjgren's syndrome may relate to both genotype and salivary gland homing.Clin Exp Immunol. 2018 Jun;192(3):259-270. doi: 10.1111/cei.13118. Epub 2018 Mar 24.
36 Aromatase controls Sjgren syndrome-like lesions through monocyte chemotactic protein-1 in target organ and adipose tissue-associated macrophages.Am J Pathol. 2015 Jan;185(1):151-61. doi: 10.1016/j.ajpath.2014.09.006. Epub 2014 Oct 31.
37 Unexpected development of autoimmunity in BAFF-R-mutant MRL-lpr mice.Immunology. 2007 Feb;120(2):281-9. doi: 10.1111/j.1365-2567.2006.02500.x. Epub 2006 Oct 31.
38 Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjgren's syndrome.Nat Genet. 2013 Nov;45(11):1284-92. doi: 10.1038/ng.2792. Epub 2013 Oct 6.
39 Identification of a quantitative trait locus regulating B cell-dominant infiltration into autoimmune sialitis lesions of the IQI mouse model of primary Sjgren's syndrome.Immunogenetics. 2007 Nov;59(11):853-9. doi: 10.1007/s00251-007-0244-4. Epub 2007 Oct 16.
40 Rapamycin Eye Drops Suppress Lacrimal Gland Inflammation In a Murine Model of Sjgren's Syndrome.Invest Ophthalmol Vis Sci. 2017 Jan 1;58(1):372-385. doi: 10.1167/iovs.16-19159.
41 Reduced Corneal Innervation in the CD25 Null Model of Sjgren Syndrome.Int J Mol Sci. 2018 Nov 30;19(12):3821. doi: 10.3390/ijms19123821.
42 Interleukin-21-mediated suppression of the Pax3-Id3 pathway exacerbates the development of Sjgren's syndrome via follicular helper T cells.Cytokine. 2020 Jan;125:154834. doi: 10.1016/j.cyto.2019.154834. Epub 2019 Sep 3.
43 Act1 is a negative regulator in T and B cells via direct inhibition of STAT3.Nat Commun. 2018 Jul 16;9(1):2745. doi: 10.1038/s41467-018-04974-3.
44 Leptin/OB-R pathway promotes IL-4 secretion from B lymphocytes and induces salivary gland epithelial cell apoptosis in Sjgren's syndrome.Oncotarget. 2017 Jun 28;8(38):63417-63429. doi: 10.18632/oncotarget.18823. eCollection 2017 Sep 8.
45 Anti-IL-7 receptor- treatment ameliorates newly established Sjgren's-like exocrinopathy in non-obese diabetic mice.Biochim Biophys Acta Mol Basis Dis. 2018 Jul;1864(7):2438-2447. doi: 10.1016/j.bbadis.2018.04.010. Epub 2018 Apr 19.
46 Tissue-Resident Memory CD8+ T Cells Acting as Mediatorsof Salivary Gland Damage in a Murine Model of Sjgren's Syndrome.Arthritis Rheumatol. 2019 Jan;71(1):121-132. doi: 10.1002/art.40676. Epub 2018 Dec 4.
47 Suppression of Th1-Mediated Keratoconjunctivitis Sicca by Lifitegrast.J Ocul Pharmacol Ther. 2018 Sep;34(7):543-549. doi: 10.1089/jop.2018.0047. Epub 2018 Jun 29.
48 IP3R deficit underlies loss of salivary fluid secretion in Sjgren's Syndrome.Sci Rep. 2015 Sep 14;5:13953. doi: 10.1038/srep13953.
49 Dual function of miR-1248 links interferon induction and calcium signaling defects in Sjgren's syndrome.EBioMedicine. 2019 Oct;48:526-538. doi: 10.1016/j.ebiom.2019.09.010. Epub 2019 Oct 6.
50 Functional up-regulation of HERG K+ channels in neoplastic hematopoietic cells.J Biol Chem. 2002 May 24;277(21):18528-34. doi: 10.1074/jbc.M200592200. Epub 2002 Mar 13.
51 Genome-Wide Association Analysis Reveals Genetic Heterogeneity of Sjgren's Syndrome According to Ancestry.Arthritis Rheumatol. 2017 Jun;69(6):1294-1305. doi: 10.1002/art.40040. Epub 2017 May 9.
52 Lymphotoxin-beta receptor blockade reduces CXCL13 in lacrimal glands and improves corneal integrity in the NOD model of Sjgren's syndrome.Arthritis Res Ther. 2011;13(6):R182. doi: 10.1186/ar3507. Epub 2011 Nov 1.
53 Ly9 (CD229) Antibody Targeting Depletes Marginal Zone and Germinal Center B Cells in Lymphoid Tissues and Reduces Salivary Gland Inflammation in a Mouse Model of Sjgren's Syndrome.Front Immunol. 2018 Nov 16;9:2661. doi: 10.3389/fimmu.2018.02661. eCollection 2018.
54 Novel Sjgren's autoantibodies found in fibromyalgia patients with sicca and/or xerostomia.Autoimmun Rev. 2019 Feb;18(2):199-202. doi: 10.1016/j.autrev.2018.09.004. Epub 2018 Dec 18.
55 Aqueous Tear Deficiency Increases Conjunctival Interferon- (IFN-) Expression and Goblet Cell Loss.Invest Ophthalmol Vis Sci. 2015 Nov;56(12):7545-50. doi: 10.1167/iovs.15-17627.
56 Characteristics of Labial Gland Mesenchymal Stem Cells of Healthy Individuals and Patients with Sjgren's Syndrome: A Preliminary Study.Stem Cells Dev. 2017 Aug 15;26(16):1171-1185. doi: 10.1089/scd.2017.0045. Epub 2017 Jun 26.
57 A missense variant in NCF1 is associated with susceptibility to multiple autoimmune diseases.Nat Genet. 2017 Mar;49(3):433-437. doi: 10.1038/ng.3782. Epub 2017 Jan 30.
58 Defective feedback regulation of NF-kappaB underlies Sjogren's syndrome in mice with mutated kappaB enhancers of the IkappaBalpha promoter.Proc Natl Acad Sci U S A. 2010 Aug 24;107(34):15193-8. doi: 10.1073/pnas.1005533107. Epub 2010 Aug 9.
59 Myoepithelial cell-driven acini contraction in response to oxytocin receptor stimulation is impaired in lacrimal glands of Sjgren's syndrome animal models.Sci Rep. 2018 Jul 2;8(1):9919. doi: 10.1038/s41598-018-28227-x.
60 Degradation of proteoglycan 4/lubricin by cathepsin S: Potential mechanism for diminished ocular surface lubrication in Sjgren's syndrome.Exp Eye Res. 2017 Aug;161:1-9. doi: 10.1016/j.exer.2017.05.006. Epub 2017 May 23.
61 Expression of proteasomal immunosubunit beta1i is dysregulated in inflammatory infiltrates of minor salivary glands in Sjogren's syndrome.J Rheumatol. 2009 Dec;36(12):2694-703. doi: 10.3899/jrheum.081098. Epub 2009 Oct 15.
62 Autoantibodies to the amino-terminal fragment of beta-fodrin expressed in glandular epithelial cells in patients with Sjgren's syndrome.J Immunol. 2001 Nov 1;167(9):5449-56. doi: 10.4049/jimmunol.167.9.5449.
63 Association of High Mobility Group Box Chromosomal Protein 1 and Receptor for Advanced Glycation End Products Serum Concentrations With Extraglandular Involvement and Disease Activity in Sjgren's Syndrome.Arthritis Care Res (Hoboken). 2018 Jun;70(6):944-948. doi: 10.1002/acr.23420. Epub 2018 Apr 23.
64 Matriptase deletion initiates a Sjgren's syndrome-like disease in mice.PLoS One. 2014 Feb 13;9(2):e82852. doi: 10.1371/journal.pone.0082852. eCollection 2014.
65 Dysregulated Marginal Zone B Cell Compartment in a Mouse Model of Sjgren's Syndrome with Ocular Inflammation.Int J Mol Sci. 2018 Oct 11;19(10):3117. doi: 10.3390/ijms19103117.
66 Abundant a proliferation-inducing ligand (APRIL)-producing macrophages contribute to plasma cell accumulation in immunoglobulin G4-related disease.Nephrol Dial Transplant. 2019 Jun 1;34(6):960-969. doi: 10.1093/ndt/gfy296.
67 Molecular Evidence for Precursors of Sjgren's Foci in Histologically Normal Lacrimal Glands.Int J Mol Sci. 2019 Jan 8;20(1):223. doi: 10.3390/ijms20010223.
68 Interleukin-25 Axis Is Involved in the Pathogenesis of Human Primary and Experimental Murine Sjgren's Syndrome.Arthritis Rheumatol. 2018 Aug;70(8):1265-1275. doi: 10.1002/art.40500. Epub 2018 Jun 27.
69 A 1.1-Mb transcript map of the hereditary hemochromatosis locus.Genome Res. 1997 May;7(5):441-56. doi: 10.1101/gr.7.5.441.
70 Immunoglobulin VH genes in thymic MALT lymphoma are biased toward a restricted repertoire and are frequently unmutated.J Pathol. 2006 Feb;208(3):415-22. doi: 10.1002/path.1889.
71 Monocytes from Sjgren's syndrome patients display increased vasoactive intestinal peptide receptor 2 expression and impaired apoptotic cell phagocytosis.Clin Exp Immunol. 2014 Sep;177(3):662-70. doi: 10.1111/cei.12378.
72 Homozygosity for the 168His variant of the minor histocompatibility antigen HA-1 is associated with reduced risk of primary Sjgren's syndrome.Eur J Immunol. 2005 Jan;35(1):305-17. doi: 10.1002/eji.200425406.
73 Healthy human salivary glands contain a DHEA-sulphate processing intracrine machinery, which is deranged in primary Sjgren's syndrome.J Cell Mol Med. 2009 Jul;13(7):1261-70. doi: 10.1111/j.1582-4934.2009.00727.x. Epub 2009 Feb 27.
74 Th17/Treg cell level and clinical characteristics of peripheral blood of patients with Sjogren's syndrome complicated with primary biliary cirrhosis.Medicine (Baltimore). 2019 Jun;98(24):e15952. doi: 10.1097/MD.0000000000015952.
75 NUDT15 R139C variation increases the risk of azathioprine-induced toxicity in Chinese subjects: Case report and literature review.Medicine (Baltimore). 2018 Apr;97(17):e0301. doi: 10.1097/MD.0000000000010301.
76 Variants at potential loci associated with Sjogren's syndrome in Koreans: A genetic association study.Clin Immunol. 2019 Oct;207:79-86. doi: 10.1016/j.clim.2019.07.010. Epub 2019 Jul 23.
77 Anti-FIRexon2, a splicing variant form of PUF60, autoantibody is detected in the sera of esophageal squamous cell carcinoma.Cancer Sci. 2019 Jun;110(6):2004-2013. doi: 10.1111/cas.14024. Epub 2019 May 20.
78 Small proline-rich protein 1B (SPRR1B) is a biomarker for squamous metaplasia in dry eye disease.Invest Ophthalmol Vis Sci. 2008 Jan;49(1):34-41. doi: 10.1167/iovs.07-0685.
79 Elevation of autophagy markers in Sjgren syndrome dry eye.Sci Rep. 2017 Dec 8;7(1):17280. doi: 10.1038/s41598-017-17128-0.
80 TREX1 variants in Sjogren's syndrome related lymphomagenesis.Cytokine. 2020 Aug;132:154781. doi: 10.1016/j.cyto.2019.154781. Epub 2019 Jul 18.
81 T-Cell-Specific PTPN2 Deficiency in NOD Mice Accelerates the Development of Type 1 Diabetes and Autoimmune Comorbidities.Diabetes. 2019 Jun;68(6):1251-1266. doi: 10.2337/db18-1362. Epub 2019 Apr 1.
82 Ablation of the Chaperone Protein ERdj5 Results in a Sjgren's Syndrome-Like Phenotype in Mice, Consistent With an Upregulated Unfolded Protein Response in Human Patients.Front Immunol. 2019 Mar 22;10:506. doi: 10.3389/fimmu.2019.00506. eCollection 2019.
83 A rare case of autoimmune polyglandular syndrome with Sjgren's syndrome and primary hypoparathyroidism.BMJ Case Rep. 2019 May 27;12(5):e228634. doi: 10.1136/bcr-2018-228634.
84 Aire-deficient mice provide a model of corneal and lacrimal gland neuropathy in Sjgren's syndrome.PLoS One. 2017 Sep 19;12(9):e0184916. doi: 10.1371/journal.pone.0184916. eCollection 2017.
85 Syringomyelia-like syndrome in neuromyelitis optica spectrum disorder complicated with Sjogren's syndrome: a case report.BMC Neurol. 2018 Oct 9;18(1):168. doi: 10.1186/s12883-018-1170-9.
86 Blockade of CD40-CD154 pathway interactions suppresses ectopic lymphoid structures and inhibits pathology in the NOD/ShiLtJ mouse model of Sjgren's syndrome.Ann Rheum Dis. 2019 Jul;78(7):974-978. doi: 10.1136/annrheumdis-2018-213929. Epub 2019 Mar 22.
87 Achaete-Scute Homologue 2-Regulated Follicular Helper T Cells Promote Autoimmunity in a Murine Model for Sjgren Syndrome.Am J Pathol. 2019 Dec;189(12):2414-2427. doi: 10.1016/j.ajpath.2019.08.008. Epub 2019 Sep 17.
88 Primary Sjgren's syndrome.Lupus. 2018 Oct;27(1_suppl):32-35. doi: 10.1177/0961203318801673.
89 Clinical expression of systemic lupus erythematosus in patients with C4A deficiency.Medicine (Baltimore). 1993 Jul;72(4):236-44. doi: 10.1097/00005792-199307000-00003.
90 Pathogenesis of IgG4-related disease. Comparison with Sjgren's syndrome.Mod Rheumatol. 2020 Jan;30(1):7-16. doi: 10.1080/14397595.2019.1650694. Epub 2019 Aug 19.
91 Retrovirus in salivary glands from patients with Sjgren's syndrome.J Clin Pathol. 1997 Mar;50(3):223-30. doi: 10.1136/jcp.50.3.223.
92 Brief Report: Anti-Calponin 3 Autoantibodies: A Newly Identified Specificity in Patients With Sjgren's Syndrome.Arthritis Rheumatol. 2018 Oct;70(10):1610-1616. doi: 10.1002/art.40550. Epub 2018 Sep 3.
93 Androgen deficiency and defective intracrine processing of dehydroepiandrosterone in salivary glands in Sjgren's syndrome.J Rheumatol. 2008 Nov;35(11):2229-35. doi: 10.3899/jrheum.080220. Epub 2008 Oct 1.
94 "Lymphoid" chemokine messenger RNA expression by epithelial cells in the chronic inflammatory lesion of the salivary glands of Sjgren's syndrome patients: possible participation in lymphoid structure formation.Arthritis Rheum. 2001 Feb;44(2):408-18. doi: 10.1002/1529-0131(200102)44:2<408::AID-ANR60>3.0.CO;2-0.
95 Activation of Peptidylarginine Deiminase in the Salivary Glands of Balb/c Mice Drives the Citrullination of Ro and La Ribonucleoproteins.J Immunol Res. 2017;2017:8959687. doi: 10.1155/2017/8959687. Epub 2017 Nov 26.
96 Association between three autoimmune diseases: vitiligo, primary biliary cirrhosis, and Sjgren's syndrome.An Bras Dermatol. 2019 Nov-Dec;94(6):710-712. doi: 10.1016/j.abd.2018.07.002. Epub 2019 Oct 23.
97 Increased expression of human thioredoxin/adult T cell leukemia-derived factor in Sjgren's syndrome.Arthritis Rheum. 1996 May;39(5):773-82. doi: 10.1002/art.1780390509.
98 Downstream activation of NF-B in the EDA-A1/EDAR signalling in Sjgren's syndrome and its regulation by the ubiquitin-editing enzyme A20.Clin Exp Immunol. 2016 May;184(2):183-96. doi: 10.1111/cei.12764. Epub 2016 Feb 23.
99 The expression of human endogenous retrovirus-3 in fetal cardiac tissue and antibodies in congenital heart block.Clin Exp Immunol. 1996 Jun;104(3):388-93.
100 Plasma -L-fucosidase-1 in patients with Sjgren's syndrome and other rheumatic disorders.Int J Rheum Dis. 2019 Sep;22(9):1762-1767. doi: 10.1111/1756-185X.13639. Epub 2019 Aug 16.
101 Comparison of telomere length and association with progenitor cell markers in lacrimal gland between Sjgren syndrome and non-Sjgren syndrome dry eye patients.Mol Vis. 2011;17:1397-404. Epub 2011 May 27.
102 Molecular cloning of a novel 97-kd Golgi complex autoantigen associated with Sjgren's syndrome.Arthritis Rheum. 1997 Sep;40(9):1693-702. doi: 10.1002/art.1780400920.
103 Novel variant of p230 trans-Golgi network protein identified by serum from Sjgren's syndrome patient.Eur J Cell Biol. 2000 Nov;79(11):790-4. doi: 10.1078/0171-9335-00114.
104 Detection of Grb-2-related adaptor protein gene (GRAP) and peptide molecule in salivary glands of MRL/lpr mice and patients with Sjgren's syndrome.J Int Med Res. 2004 May-Jun;32(3):284-91. doi: 10.1177/147323000403200308.
105 Identification of susceptibility gene associated with female primary Sjgren's syndrome in Han Chinese by genome-wide association study.Hum Genet. 2016 Nov;135(11):1287-1294. doi: 10.1007/s00439-016-1716-0. Epub 2016 Aug 8.
106 Lesional CD4+ IFN-+ cytotoxic T lymphocytes in IgG4-related dacryoadenitis and sialoadenitis.Ann Rheum Dis. 2017 Feb;76(2):377-385. doi: 10.1136/annrheumdis-2016-209139. Epub 2016 Jun 29.
107 Evaluation of the corneal epithelium in non-Sjgren's and Sjgren's dry eyes: an in vivo confocal microscopy study using HRT III RCM.BMC Ophthalmol. 2018 Dec 4;18(1):309. doi: 10.1186/s12886-018-0971-3.
108 Associations of MHC class II alleles in Norwegian primary Sjgren's syndrome patients: implications for development of autoantibodies to the Ro52 autoantigen.Scand J Immunol. 2001 Oct;54(4):428-33. doi: 10.1046/j.1365-3083.2001.00993.x.
109 Fibulin-6 expression and anoikis in human salivary gland epithelial cells: implications in Sjogren's syndrome.Int Immunol. 2009 Mar;21(3):303-11. doi: 10.1093/intimm/dxp001. Epub 2009 Feb 3.
110 CP-25 Alleviates Experimental Sjgren's Syndrome Features in NOD/Ltj Mice and Modulates T Lymphocyte Subsets.Basic Clin Pharmacol Toxicol. 2018 Oct;123(4):423-434. doi: 10.1111/bcpt.13025. Epub 2018 Jun 1.
111 IFI16 filament formation in salivary epithelial cells shapes the anti-IFI16 immune response in Sjgren's syndrome.JCI Insight. 2018 Sep 20;3(18):e120179. doi: 10.1172/jci.insight.120179. eCollection 2018 Sep 20.
112 Expression of type III interferons (IFNs) and their receptor in Sjgren's syndrome.Clin Exp Immunol. 2016 Dec;186(3):304-312. doi: 10.1111/cei.12865. Epub 2016 Oct 4.
113 Local and Systemic IKK and NF-B Signaling Associated with Sjgren's Syndrome Immunopathogenesis.J Immunol Res. 2015;2015:534648. doi: 10.1155/2015/534648. Epub 2015 Aug 26.
114 First Association of Interleukin 12 Receptor Beta 1 Deficiency with Sjgren's Syndrome.Front Immunol. 2017 Jul 28;8:885. doi: 10.3389/fimmu.2017.00885. eCollection 2017.
115 ILF2 and ILF3 are autoantigens in canine systemic autoimmune disease.Sci Rep. 2018 Mar 19;8(1):4852. doi: 10.1038/s41598-018-23034-w.
116 Expression of the immune regulator tripartite-motif 21 is controlled by IFN regulatory factors.J Immunol. 2013 Oct 1;191(7):3753-63. doi: 10.4049/jimmunol.1202341. Epub 2013 Aug 23.
117 Myeloid calcium binding proteins: expression in the differentiated HL-60 cells and detection in sera of patients with connective tissue diseases.J Biochem. 1990 Oct;108(4):650-3. doi: 10.1093/oxfordjournals.jbchem.a123257.
118 Transcriptional and metabolic pre-B cell receptor-mediated checkpoints: implications for autoimmune diseases.Mol Immunol. 2014 Dec;62(2):315-20. doi: 10.1016/j.molimm.2014.01.009. Epub 2014 Mar 4.
119 Identification of tear lipocalin as a novel autoantigen target in Sjgren's syndrome.J Autoimmun. 2005 Nov;25(3):229-34. doi: 10.1016/j.jaut.2005.09.021. Epub 2005 Oct 24.
120 Genetic contributors and soluble mediators in prediction of autoimmune comorbidity.J Autoimmun. 2019 Nov;104:102317. doi: 10.1016/j.jaut.2019.102317. Epub 2019 Aug 20.
121 MUC19 expression in human ocular surface and lacrimal gland and its alteration in Sjgren syndrome patients.Exp Eye Res. 2008 Feb;86(2):403-11. doi: 10.1016/j.exer.2007.11.013. Epub 2007 Nov 28.
122 Reduced sulfation of muc5b is linked to xerostomia in patients with Sjgren syndrome.Ann Rheum Dis. 2008 Oct;67(10):1480-7. doi: 10.1136/ard.2007.078246. Epub 2007 Nov 12.
123 Mesenchymal stem cell transplantation alleviates experimental Sjgren's syndrome through IFN-/IL-27 signaling axis.Theranostics. 2019 Oct 21;9(26):8253-8265. doi: 10.7150/thno.37351. eCollection 2019.
124 When killers become helpers.Sci Transl Med. 2013 Jul 24;5(195):195fs29. doi: 10.1126/scitranslmed.3006850.
125 The IkappaBL gene polymorphism influences risk of acquiring systemic lupus erythematosus and Sjgren's syndrome.Hum Immunol. 2008 Jan;69(1):45-51. doi: 10.1016/j.humimm.2007.11.008. Epub 2007 Dec 26.
126 Prevalence of anti-NT5C1A antibodies in Japanese patients with autoimmune rheumatic diseases in comparison with other patient cohorts.Clin Chim Acta. 2017 Sep;472:1-4. doi: 10.1016/j.cca.2017.07.002. Epub 2017 Jul 4.
127 Publisher Correction: A functional variant in the OAS1 gene is associated with Sjgren's syndrome complicated with HBV infection.Sci Rep. 2018 Nov 14;8(1):17031. doi: 10.1038/s41598-018-35438-9.
128 Tissue-specific up-regulation of the proteasome subunit beta5i (LMP7) in Sjgren's syndrome.Arthritis Rheum. 2006 May;54(5):1501-8. doi: 10.1002/art.21782.
129 Changes in Rab3D expression and distribution in the acini of Sjgren's syndrome patients are associated with loss of cell polarity and secretory dysfunction.Arthritis Rheum. 2011 Oct;63(10):3126-35. doi: 10.1002/art.30500.
130 The immune response to and expression of cross-reactive retroviral gag sequences in autoimmune disease.Br J Rheumatol. 1992 Nov;31(11):735-42. doi: 10.1093/rheumatology/31.11.735.
131 Immunotherapeutic targets in estrogen deficiency-dependent Sjgren's syndrome-related manifestations.Immunotherapy. 2010 May;2(3):339-46. doi: 10.2217/imt.10.18.
132 In primary Sjgren's syndrome, HLA class II is associated exclusively with autoantibody production and spreading of the autoimmune response.Arthritis Rheum. 2003 Aug;48(8):2240-5. doi: 10.1002/art.11103.
133 A new overgrowth syndrome is due to mutations in RNF125. Hum Mutat. 2014 Dec;35(12):1436-41. doi: 10.1002/humu.22689.
134 Hypocomplementaemic urticarial vasculitis, angio-oedema and 'lupus-like' disease: association with C4B null allele.Clin Exp Rheumatol. 1987 Apr-Jun;5(2):161-4.
135 Ro60 and Y RNAs: structure, functions, and roles in autoimmunity.Crit Rev Biochem Mol Biol. 2019 Apr;54(2):133-152. doi: 10.1080/10409238.2019.1608902. Epub 2019 May 14.
136 Decreased Expression of Semaphorin 3A and Semaphorin 7A Levels and Its Association with Systemic Lupus Erythematosus.Immunol Invest. 2020 Feb;49(1-2):69-80. doi: 10.1080/08820139.2019.1649280. Epub 2019 Aug 15.
137 Clinical and immunological characteristics of patients with Sjgren's syndrome in relation to alpha-fodrin antibodies.Rheumatology (Oxford). 2007 Mar;46(3):479-83. doi: 10.1093/rheumatology/kel270. Epub 2006 Aug 25.
138 Antigen-driven selection of antibodies against SSA, SSB and the centromere 'complex', including a novel antigen, MIS12 complex, in human salivary glands.Ann Rheum Dis. 2020 Jan;79(1):150-158. doi: 10.1136/annrheumdis-2019-215862. Epub 2019 Oct 14.
139 Association of the TAP2*Bky2 allele with presence of SS-A/Ro and other autoantibodies in Japanese patients with systemic lupus erythematosus.Lupus. 2003;12(4):258-65. doi: 10.1191/0961203303lu344oa.
140 Initial human T-cell leukemia virus type 1 infection of the salivary gland epithelial cells requires a biofilm-like structure.Virus Res. 2019 Aug;269:197643. doi: 10.1016/j.virusres.2019.197643. Epub 2019 Jun 21.
141 Autoantibodies against the Immunoglobulin-Binding Region of Ro52 Link its Autoantigenicity with Pathogen Neutralization.Sci Rep. 2018 Feb 20;8(1):3345. doi: 10.1038/s41598-018-21522-7.
142 SS-56, a novel cellular target of autoantibody responses in Sjgren syndrome and systemic lupus erythematosus.J Clin Invest. 2001 Sep;108(6):861-9. doi: 10.1172/JCI13469.
143 X-linked ectodermal dysplasia receptor (XEDAR) gene silencing prevents caspase-3-mediated apoptosis in Sjgren's syndrome.Clin Exp Med. 2017 Feb;17(1):111-119. doi: 10.1007/s10238-015-0404-z. Epub 2015 Dec 11.