U1snRNPU1smallnuclearribonucleoproteinHSPheatshockproteinhnRNPheterogenousnuclearribonucleoproteinTCRTcellreceptorVvariableregionofchainAVvariableregionofchainCDRcomplementarydeterminin ID: 942800
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Bothanti-ECantibodyandendothelin-1havebeenconsideredtoparticipateintheprocessofvasculardamageinMCTDpatients.Theantibodytospliceo(A2/hnRNP)wasfirstdescribedbyHassfeldetal.(1995)intheseraof38%
ofpatientswithMCTD,andthisantibodywasconsideredtointeractwithhnRNPproteins.Thisantibodytoheterogeneousnuclearribonucleoprocomplex(hnRNP-A2)wasfoundatalmostanequivalentfrequencyinMixed Connec
tive Tissue Disease247A.Autoantibodies toFrequencyReferenceU1snRNP100%Burdt et al. 1999new epitope combined no kDa100%Murakami et al. 2002TS1-RNA31,7%Ikeda et al. 200373 kDa HSP100%Mairesse
et al. 1993Casein kinase15%Wiemann et al. 1993Endothelial cells57%Filep et al. 1995Spliceosome (A2/hn RNP)38%Hassfeld et al. 1995Human endogenous retrovirus p30gag33%Hishikawa et al. 1997Pho
spholipid15%Komatireddy et al. 1997Fibrillin-134%Tan et al. 1999Nuclear matrix100%Sato et al. 2000B.T cell characteristicsCD4+CD45+RAincreasedBecker et al. 1992decreasedIkaeheimo et al. 1994
1, 3, 4, 5.2, 14, 16frequently usedOkubo et al. 1994TCR AV CDR3 (common motif)highly conservedTalken et al. 1999C.HLA class IIDR4in AmericanincreasedHoffmann et al. 1990DR4 (B15, DR4)in Finn
ishincreasedRuuska et al. 1992DR2 or DR4in English (UCTD)increasedGendi et al. 1995DRB4* 0101,in JapaneseincreasedDong et al. 1993DQA1* 03(RR = 2.47, 2.25)DQB1* 0501in MexicanincreasedWeckma
nn et al. 1999 U1snRNP:U1smallnuclearribonucleoprotein,HSP:heatshockprotein,hnRNP:heterogenousnuclearribonucleoprotein,TCR:Tcellreceptor,V:variableregionof-chain,AV:variableregionof-chain,CD
R:complementarydeterminingregion,UCTD:undifferentia Mixed Connective Tissue Disease251Fig.2.Faceofa32-year-oldwomanwithmixedconnectivetissuedisease.Discretefacial Mixed Connective Tissue Dis
ease253Table3.Characteristicsof3SetsofCriteriafortheClassificationofMixedConnectiveTissueReferenceCriteriaRequirements for diagnosisAlarcon-SegoviaA.SerologicSerological criterion plusand Vi
llarreal1.Anti-RNP at a hemagglutinationat least 3 clinical criteria, or(1987)titer of1:1,600including either synovitisB.Clinicalmyositis.1.Edema in the hands2.Synovitis3.Myositis4.Raynauds
phenomenon5.AcrosclerosisKasukawa et al.A.Common symptomsAt least 1 of the 2 common(1987)1.Raynauds phenomenonsymptoms plus positive for2.Swollen fingers or handsanti-snRNP plus 1 or moreB
.Anti-snRNP antibody positiveof the mixed symptoms inC.Mixed symptomsat least 2 of the 3 disease1.SLE-like findingscategories.a.Polyarthritisb.Lymphadenopathyc.Facial erythemad.Pericarditis
or pleuritise.Leuko- or thrombocytopenia2.SSc-like findingsa.Sclerodactylyb.Pulmonary fibrosis, restrictivec.Hypomotility or dilatation of3.PM-like findingsa.Muscle weaknessb.Elevated serum
levels ofc.Myogenic pattern on EMGSharp (1987)A.Major At least 4 major criteria1.Myositis, severe1:4,000 (exclusion criterion:2.Pulmonary involvementpositivity for anti-Sm);a.Diffusion capac
ity Or, 2 major criteria fromof normal valuesamong criteria 1, 2, and 3b.Pulmonary hypertensionplus 2 minor criteria plusc.Proliferative vascularanti-U1 RNP titer oflesions on lung biopsyat