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001-es BibID:	BIBFORM047802
035-os BibID:	(PMID)23637328 (WoS)000321993800017 (Scopus)84879955753
Első szerző:	Hajas Ágota Helga (orvos)
Cím:	Clinical course, prognosis, and causes of death in mixed connective tissue disease / Agota Hajas, Peter Szodoray, Britt Nakken, Janos Gaal, Eva Zöld, Renata Laczik, Nora Demeter, Gabor Nagy, Zoltan Szekanecz, Margit Zeher, Gyula Szegedi, Edit Bodolay
Dátum:	2013
ISSN:	0315-162X
Megjegyzések:	To study the survival rate and prognostic indicators of mixed connective tissue disease (MCTD) in a Hungarian population. METHODS: Two hundred eighty patients with MCTD diagnosed between 1979 and 2011 were followed prospectively. Clinical features, autoantibodies, and mortality data were assessed. Prognostic factors for survival were investigated and survival was calculated from the time of the diagnosis by Kaplan-Meier method. RESULTS: A total of 22 of 280 patients died: the causes of death were pulmonary arterial hypertension (PAH) in 9 patients, thrombotic thrombocytopenic purpura in 3, infections in 3, and cardiovascular events in 7. The 5, 10, and 15-year survival rates after the diagnosis was established were 98%, 96%, and 88%, respectively. The deceased patients were younger at the diagnosis of MCTD compared to patients who survived (35.5 ± 10.4 vs 41.8 ± 10.7 yrs; p < 0.03), while there was no difference in the duration of the disease (p = 0.835). Our cohort study showed that the presence of cardiovascular events (p < 0.0001), esophageal hypomotility (p = 0.04), serositis (p < 0.001), secondary antiphospholipid syndrome (p = 0.039), and malignancy (p < 0.001) was significantly higher in the deceased patients with MCTD. The presence of anticardiolipin (p = 0.019), anti-β2-glycoprotein I (p = 0.002), and antiendothelial cell antibodies (p = 0.002) increased the risk of mortality. CONCLUSION: Overall, PAH remained the leading cause of death in patients with MCTD. The prevalence of cardiovascular morbidity and mortality, malignancy, and thrombotic events increased during the disease course of MCTD. The presence of antiphospholipid antibodies raised the risk of mortality.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk
Megjelenés:	Journal of Rheumatology. - 40 : 7 (2013), p. 1134-1142. -
További szerzők:	Szodoray Péter (1973-) (belgyógyász, orvos) Nakken, Britt Gaál János (1965-) (reumatológus, belgyógyász) Zöld Éva (1978-) (belgyógyász) Laczik Renáta (1982-) (orvos) Demeter Nóra Nagy Gábor (1974-) (laboratóriumi szakorvos, laboratóriumi hematológus és immunológus) Szekanecz Zoltán (1964-) (reumatológus, belgyógyász, immunológus) Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Szegedi Gyula (1936-2013) (belgyógyász, immunológus) Bodolay Edit (1950-) (belgyógyász, allergológus és klinikai immunológus)
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001-es BibID:	BIBFORM046547
035-os BibID:	PMID:23608739
Első szerző:	Hajas Ágota Helga (orvos)
Cím:	Derailed B cell homeostasis in patients with mixed connective tissue disease / A. Hajas, S. Barath, P. Szodoray, B. Nakken, P. Gogolak, Z. Szekanecz, E. Zold, M. Zeher, G. Szegedi, E. Bodolay
Dátum:	2013
ISSN:	0198-8859
Megjegyzések:	Mixed connective tissue disease (MCTD) is a systemic autoimmune disorder, characterized by the presence of antibodies to U1-RNP protein. We aimed to determine phenotypic abnormalities of peripheral B cell subsets in MCTD. Blood samples were obtained from 46 MCTD patients, and 20 controls. Using anti-CD19, anti-CD27, anti-IgD and anti-CD38 monoclonal antibodies, the following B cell subsets were identified by flow cytometry: (1) transitional B cells (CD19+CD27-IgD+CD38(high)); (2) naive B cells (CD19+CD27-IgD+CD38(low)); (3) non-switched memory B cells (CD19+CD27+IgD+); (4) switched memory B cells (CD19+CD27+IgD-); (5) double negative (DN) memory B cells (CD19+CD27-IgD-) and (6) plasma cells (CD19+CD27(high)IgD-). The proportion of transitional B cells, naive B cells and DN B lymphocytes was higher in MCTD than in controls. The DN B cells were positive for CD95 surface marker. This memory B cells population showed a close correlation with disease activity. The number of plasma cells was also increased, and there was an association between the number of plasma cells and the anti-U1RNP levels. Cyclophosphamide, methotrexate, and corticosteroid treatment decreased the number of DN and CD27(high) B cells. In conclusion, several abnormalities were found in the peripheral B-cell subsets in MCTD, which reinforces the role of derailed humoral autoimmune processes in the pathogenesis.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	Human Immunology. - 74 : 7 (2013), p. 833-841. -
További szerzők:	Baráth Sándor (1977-) (biológus) Szodoray Péter (1973-) (belgyógyász, orvos) Nakken, Britt Gogolák Péter (1968-) (biológus, immunológus) Szekanecz Zoltán (1964-) (reumatológus, belgyógyász, immunológus) Zöld Éva (1978-) (belgyógyász) Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Szegedi Gyula (1936-2013) (belgyógyász, immunológus) Bodolay Edit (1950-) (belgyógyász, allergológus és klinikai immunológus)
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