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Anti U3RNP antibodies

Ewa Wielosz, Magdalena Dryglewska, Maria Majdan
INTRODUCTION: The systemic sclerosis-associated autoantibodies include anti-centromere, anti-topoisomerase I (anti-topo I), anti-RNA polymerase III, anti-fibrillarin, anti-Th/To, and anti-PDGFR. A specific serological profile is connected with clinical manifestations and prognosis in systemic sclerosis (SSc). OBJECTIVES: The aim of the study was to assess the serological profile in limited cutaneous and diffuse cutaneous SSc (lcSSc and dcSSc). PATIENTS AND METHODS: 87 (68 female and 19 male) consecutive SSc patients treated between 2006 and 2011 were assessed...
August 18, 2014: Postȩpy Higieny i Medycyny Doświadczalnej
Emma C Derrett-Smith, Svetlana I Nihtyanova, Jennifer Harvey, Alan D Salama, Christopher P Denton
OBJECTIVES: To define the clinical, serological, histological and immunogenetic features of patients with scleroderma and ANCA-associated vasculitis (AAV). METHODS: We examined a clinical database of 2,200 patients with either limited or diffuse cutaneous systemic sclerosis (SSc). Patients with a confirmed diagnosis of vasculitis who were ANCA positive with either MPO or PR3 reactivity had their clinical features, serology, histology and HLA haplotypes examined in detail...
October 2013: Rheumatology
Yasuhito Hamaguchi
Systemic sclerosis (SSc) is a connective tissue disorder characterized by microvascular damage and excessive fibrosis of the skin and internal organs with autoimmune background. One representative feature of the immunological abnormalities in SSc patients is the presence of antinuclear antibodies (ANA). More than 90% of patients with SSc are positive for ANA. Although a role of ANA in the pathogenesis in SSc remains unclear, the particular ANA are often indicative of clinical feature, disease course, and overall severity...
2013: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Angela Ceribelli, Minoru Satoh, Edward K L Chan
INTRODUCTION: Classic anti-nucleolar antibodies anti-Th/To and U3 ribonucleoprotein (-U3RNP) can help in the diagnosis, prediction of organ involvement and prognosis in systemic sclerosis (SSc); however, no validated commercial assay is available. We aimed at establishing a novel quantitative real time PCR (qPCR) method to detect these antibodies. METHODS: Standard immunoprecipitation (IP) was performed using K562 cell extract and RNA components were extracted. cDNA was reverse transcribed from RNA components and Th RNA and U3 RNA were detected by qPCR using custom primers...
2012: Arthritis Research & Therapy
Malgorzata E Krzyszczak, Yi Li, Steven J Ross, Angela Ceribelli, Edward K L Chan, Michael R Bubb, Eric S Sobel, Westley H Reeves, Minoru Satoh
Autoantibodies to topoisomerase I (topo I), RNA polymerase III (RNAPIII), centromere, U3RNP/fibrillarin, Th, PM-Scl, and U1RNP found in scleroderma (SSc) are associated with unique clinical subsets. The effects of race and gender on autoantibody prevalence and clinical manifestations were examined. Autoantibodies in sera from 105 SSc (include 75 Caucasian, 24 African-American, 6 others; 89 females and 16 males) were analyzed by immunofluorescence and immunoprecipitation. Clinical information was from database...
October 2011: Clinical Rheumatology
Tsuneyo Mimori
No abstract text is available yet for this article.
June 2010: Nihon Rinsho. Japanese Journal of Clinical Medicine
Ozlem Pehlivan, Murat Inanç
Pulmonary arterial hypertension (PAH) is an important complication of connective tissue diseases (CTD) and especially seen in systemic sclerosis, systemic lupus erythematosis (SLE), and mixed connective tissue disease (MCTD). In systemic sclerosis, PAH is isolated or accompanied by interstitial lung disease and currently, a major cause of mortality. It has been shown to be developed in approximately 10% of cases and annual screening with echocardiography has been recommended. Right heart catheterization is required for definite diagnosis...
August 2010: Anadolu Kardiyoloji Dergisi: AKD, the Anatolian Journal of Cardiology
Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Paolo Airò, Angela Tincani, Roberto Cattaneo, Brad A Pauley, Edward K L Chan, Minoru Satoh
OBJECTIVE: Patients with scleroderma (systemic sclerosis; SSc) can be classified into subsets based on autoantibody profile and clinical features. Specificities such as anti-Th/To and anti-fibrillarin (U3RNP) are detectable mainly by immunoprecipitation (IP), which is not widely used in clinical laboratories. We examined the autoantibody profiles and clinical manifestations in a cohort of Italian patients with SSc, focusing on anti-Th/To and anticentromere (ACA) antibodies, associated with limited cutaneous SSc (lcSSc)...
October 2010: Journal of Rheumatology
Yasuhito Hamaguchi
Systemic sclerosis (SSc) is thought to be an autoimmune disease, as autoantibodies against a variety of extractable nuclear antigens can be detected in patient sera. Subgrouping patients based on the type of autoantibodies present can be useful in diagnosis and management. Anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (anti-topo I) are the classic autoantibodies associated with SSc. ACA are associated with limited cutaneous involvement and isolated pulmonary hypertension, whereas anti-topo I are associated with diffuse skin involvement and pulmonary fibrosis...
January 2010: Journal of Dermatology
Svetlana I Nihtyanova, Christopher P Denton
The pathogenetic role of autoantibodies in systemic sclerosis (SSc) remains unclear, but these autoantibodies have been established as strong predictors of disease outcome and the pattern of organ complications in patients with this condition. The three most frequently observed types of SSc-specific autoantibody-anti-centromere antibodies, anti-topoisomerase antibodies and anti-RNA polymerase III antibodies-are found in over 50% of patients; the presence of each is generally exclusive of the others. Although a lot less frequently observed, antibodies directed against U3RNP and Th/To are also specific for scleroderma, whereas anti-Pm/Scl, anti-Ku and anti-U1RNP antibodies are seen mainly in patients with overlap syndromes...
February 2010: Nature Reviews. Rheumatology
Lefkothea Picha, Ioannis Pakas, Apostolia Guialis, Haralampos M Moutsopoulos, Panayiotis G Vlachoyiannopoulos
The heterogeneity of autoantibody specificities occurring in sera from patients with systemic sclerosis (SSc) raised the necessity of developing various methodologies for their detection. A cohort of 150 SSc patients were selected and tested by Indirect Immunofluorescence (IIF), Counterimmunoelectrophoresis (CIE), Immunoblot (IB) using various extracts as antigenic source and RNA precipitation. By preparing a nuclear (IB-nuclear) and a metaphase chromosomal-enriched extract (IB-MC-pellet) from HeLa cells as well as a nucleolar (IB-nucleolar) and a histone (IB-histone) extract from rat liver nuclei, we assessed their sensitivity and specificity for anti-Topo I, anti-U3RNP, anti-H1, anti-snRNPs antibodies and ACA...
September 2008: Journal of Autoimmunity
Emi Nishimagi, Akiko Tochimoto, Yasushi Kawaguchi, Takashi Satoh, Masataka Kuwana, Kae Takagi, Hisae Ichida, Tokiko Kanno, Makoto Soejima, Sayumi Baba, Naoyuki Kamatani, Masako Hara
OBJECTIVE: To clarify the clinical features of patients with systemic sclerosis (SSc) who developed severe gastrointestinal tract (GIT) involvement in the early stage of the disease. METHODS: Three hundred two consecutive Japanese patients with SSc were investigated: Group 1 comprised 14 patients with severe GIT involvement (malabsorption syndrome and/or pseudo-obstruction) within 2 years of onset of SSc; group 2 consisted of all patients without severe GIT involvement (n = 288); and group 3 consisted of 117 patients without severe GIT involvement within 2 years of onset of SSc...
October 2007: Journal of Rheumatology
Tsuneyo Mimori
No abstract text is available yet for this article.
July 2005: Nihon Rinsho. Japanese Journal of Clinical Medicine
C Ferri, M Emdin, F A Storino, D Giuggioli, G Longombardo, F Greco, N Fertig, T A Medsger
Isolated pulmonary hypertension (PHT) in patients with diffuse cutaneous systemic sclerosis (dSSc) is one of the most severe complication. Here we report the case of a 22 year-old white woman with anti-U3RNP antibody-positive dSSc complicated by severe, isolated PHT successfully treated with long-term plasma exchange. This beneficial effect persisted for two years, even after plasma exchange discontinuation. This is the first observation of isolated PHT in dSSc responsive to plasma exchange therapy.
2000: Scandinavian Journal of Rheumatology
C Rizou, J P Ioannidis, E Panou-Pomonis, M Sakarellos-Daitsiotis, C Sakarellos, H M Moutsopoulos, P G Vlachoyiannopoulos
We hypothesized that B-cell epitope mapping of DNA Topoisomerase I (type-I topoisomerase, or Topo I) may define epitopes strongly associated with pulmonary interstitial fibrosis (PIF) in systemic sclerosis (SSc). B-cell epitope mapping of Topo I was performed using 63 20-mer peptides overlapping by eight residues and spanning the entire length of the Topo I sequence. These peptides, coupled to polystyrene pins, were tested for antibody binding by enzyme-linked immunosorbent assays (ELISAs) using immunoglobulin G fractions from anti-Topo I, anticentromere, anti-U3RNP-positive, and normal sera...
March 2000: American Journal of Respiratory Cell and Molecular Biology
T Mimori
No abstract text is available yet for this article.
November 1999: Nihon Rinsho. Japanese Journal of Clinical Medicine
D G Sacks, Y Okano, V D Steen, E Curtiss, L S Shapiro, T A Medsger
OBJECTIVE: To describe a group of patients with systemic sclerosis (SSc) and diffuse cutaneous (dc) involvement with isolated pulmonary hypertension (IPHT) and to compare them to 2 other SSc patient groups, i.e., one with limited cutaneous (lc) involvement with IPHT and another with dcSSc without IPHT. METHODS: The Pittsburgh Scleroderma Databank was screened to identify appropriate patients. SSc specific serum autoantibodies were determined using published methods...
April 1996: Journal of Rheumatology
M Kuwana, T Mimori, N Hama, J Kaburaki, T Okano, T Tojo
We have characterized a clinical significance of anti-U3RNP, anti-7-2RNP, anti-RNA polymerase I and anti-PM-Scl antibody, autoantibodies to nucleolar proteins detected by immunoprecipitation method in patients with systemic sclerosis (SSc). In 248 patients with SSc, anti-U3RNP antibody was positive in 9 (3.6%), anti-7-2RNP antibody was positive in 7 (2.8%) and anti-RNA polymerase I antibody was positive in 3 (1.2%). But none of 248 patients was positive for anti-PM-Scl antibody. Anti-U3RNP antibody positive SSc patients showed significantly lower frequency of joint and lung involvements, compared with anti-U3RNP antibody negative SSc patients...
February 1992: Ryūmachi. [Rheumatism]
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