Systemic scleroderma in Greece: low mortality and strong linkage with HLA-DRB1(star)1104 allele

Vlachoyiannopoulos, PG; Dafni, UG; Pakas, I; Spyropoulou-Vlachou, M; Stavropoulos-Giokas, C; Moutsopoulos, HM

dc.contributor.author	Vlachoyiannopoulos, PG	en
dc.contributor.author	Dafni, UG	en
dc.contributor.author	Pakas, I	en
dc.contributor.author	Spyropoulou-Vlachou, M	en
dc.contributor.author	Stavropoulos-Giokas, C	en
dc.contributor.author	Moutsopoulos, HM	en
dc.date.accessioned	2014-03-01T01:50:29Z
dc.date.available	2014-03-01T01:50:29Z
dc.date.issued	2000	en
dc.identifier.issn	0003-4967	en
dc.identifier.uri	https://dspace.lib.ntua.gr/xmlui/handle/123456789/26032
dc.subject.classification	Rheumatology	en
dc.subject.other	DNA TOPOISOMERASE-I	en
dc.subject.other	SCLEROSIS SCLERODERMA	en
dc.subject.other	ANTINUCLEAR ANTIBODIES	en
dc.subject.other	CLINICAL CORRELATIONS	en
dc.subject.other	JAPANESE PATIENTS	en
dc.subject.other	HLA-DR	en
dc.subject.other	ANTICENTROMERE ANTIBODIES	en
dc.subject.other	IMMUNOLOGICAL DIFFERENCES	en
dc.subject.other	RACIAL-DIFFERENCES	en
dc.subject.other	ASSOCIATIONS	en
dc.title	Systemic scleroderma in Greece: low mortality and strong linkage with HLA-DRB1(star)1104 allele	en
heal.type	journalArticle	en
heal.language	English	en
heal.publicationDate	2000	en
heal.abstract	Objective-Description of Greek patients with scleroderma with reference to (a) major organ disease, (b) autoantibodies, (c) survival rate, and (d) HLA associations. Methods-The clinical files of 254 patients were analysed retrospectively and a standardised clinical chart was completed with age at disease onset, sex, date of first and last visit, clinical and serological findings, organs affected, reasons for death, and HLA class II alleles. HLA class II alleles (DRB1, DQA1, DQB1, DPB1) were determined by polymerase chain reaction amplification using oligopeptide probes. DNA was extracted from 98 patients and 130 Greek controls. Results-124 patients (49%) had limited systemic sclerosis (ISSc), 114 (45%) had diffuse systemic sclerosis (dSSc), and 16 (6%) had overlap syndromes. Patients with dSSc, compared with ISSc, were characterised by a higher prevalence of lung disease (p=0.0011), oesophageal, heart, and peripheral vessel disease (p=0.027, p=0.0025, and p=0.012, respectively). Anticentromere antibodies (ACA) occurred exclusively in 1SSc (34%), whereas antibodies to topoisomerase I (anti-topo I) were associated with dSSc (p<0.0001). Anti-topo I were associated with interstitial pulmonary fibrosis, oesophageal and peripheral vessel disease (p=0.028, p=0.012, and p=0.01, respectively). The HLA-DRB11104 allele was associated with the disease (p<0.0001) and anti-topo I (p<0.001), whereas it was not associated with ACA serum reactivity (p<0.001). Renal disease occurred in 4% of patients with SSc. The estimated survival probability for this cohort of patients with SSc, four years after the first visit, is 94.8%. Conclusion-SSc among Greek subjects has the same pattern of organ disease as in other white populations. However, the prevalence of kidney disease is low. The HLA class II DRB11104 allele is associated with the disease, with anti-topo I, and not associated with ACA serum reactivity.	en
heal.publisher	BRITISH MED JOURNAL PUBL GROUP	en
heal.journalName	ANNALS OF THE RHEUMATIC DISEASES	en
dc.identifier.isi	ISI:000086841800008	en
dc.identifier.volume	59	en
dc.identifier.issue	5	en
dc.identifier.spage	359	en
dc.identifier.epage	367	en