31 results on '"Ceribelli, Angela"'
Search Results
2. Reference standards for the detection of anti-mitochondrial and anti-rods/rings autoantibodies.
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Calise SJ, Zheng B, Hasegawa T, Satoh M, Isailovic N, Ceribelli A, Andrade LEC, Boylan K, Cavazzana I, Fritzler MJ, de la Torre IG, Hiepe F, Kohl K, Selmi C, Shoenfeld Y, Tincani A, and Chan EKL
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- Aged, Antiviral Agents adverse effects, Antiviral Agents therapeutic use, Autoantibodies immunology, Autoimmune Diseases diagnosis, Female, Fluorescent Antibody Technique, Indirect standards, Hepatitis C drug therapy, Humans, Immunoassay standards, Immunoprecipitation, Interferon-alpha adverse effects, Interferon-alpha therapeutic use, Laboratories, Middle Aged, Reference Standards, Ribavirin adverse effects, Ribavirin therapeutic use, Autoantibodies blood, Immunoassay methods, Mitochondria immunology
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- 2018
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3. Myositis-specific autoantibodies and their association with malignancy in Italian patients with polymyositis and dermatomyositis.
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Ceribelli A, Isailovic N, De Santis M, Generali E, Fredi M, Cavazzana I, Franceschini F, Cantarini L, Satoh M, and Selmi C
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- Adolescent, Adult, Aged, Aged, 80 and over, Autoantibodies blood, Autoantibodies chemistry, Biomarkers blood, Cohort Studies, Dermatomyositis blood, Dermatomyositis complications, Female, Humans, Immunoprecipitation, Inflammation, Italy, Male, Middle Aged, Myositis blood, Myositis complications, Neoplasms blood, Neoplasms immunology, Polymyositis blood, Polymyositis complications, Predictive Value of Tests, RNA analysis, ROC Curve, Risk, Young Adult, Autoantibodies immunology, Dermatomyositis immunology, Myositis immunology, Neoplasms complications, Polymyositis immunology
- Abstract
This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis. In six dermatomyositis cases, we found five solid forms of cancer and one Hodgkin's lymphoma in long-term remission. Among patients with cancer-associated dermatomyositis, three were positive for anti-TIF1γ/α, two for anti-Mi-2, and one for anti-MJ/NXP-2. The strongest positivity of anti-TIF1γ was seen in two active forms of cancer, and this antibody was either negative or positive at low titers in the absence of cancer or in the 7-year remission Hodgkin's lymphoma. Four out of twenty (20 %) patients with polymyositis had solid cancer, but no specific association with autoantibodies was identified; further, none of the four cases of antisynthetase syndrome had a history of cancer. No serum myositis-associated autoantibody was observed in control sera, resulting in positive predictive value 75 %, negative predictive value 78.5 %, sensitivity 50 %, specificity 92 %, and area under the ROC curve 0.7083 for the risk of paraneoplastic DM in anti-TIF1γ/α (+) patients. Myositis-specific autoantibodies can be identified thanks to the use of immunoprecipitation, and their association with cancer is particularly clear for anti-TIF1γ/α in dermatomyositis. This association should be evaluated in a prospective study by immunoprecipitation in clinical practice.
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- 2017
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4. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy.
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Satoh M, Tanaka S, Ceribelli A, Calise SJ, and Chan EK
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- Autoantigens immunology, Biomarkers analysis, Humans, Autoantibodies immunology, Myositis immunology
- Abstract
Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP). Anti-Jo-1 is the first autoantibodies to ARS detected in 15-25 % of patients. In addition to anti-Jo-1, antibodies to seven other aminoacyl tRNA synthetases (ARS) have been reported with prevalence, usually 1-5 % or lower. Patients with any anti-ARS antibodies are associated with anti-synthetase syndrome characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon, and others. Several recent studies suggested heterogeneity in clinical features among different anti-ARS antibody-positive patients and anti-ARS may also be found in idiopathic ILD without myositis. Anti-Mi-2 is a classic marker for DM and associated with good response to steroid treatment and good prognosis. Anti-SRP is specific for PM and associated with treatment-resistant myopathy histologically characterized as necrotizing myopathy. In addition to classic MSAs, several new autoantibodies with strong clinical significance have been described in DM. Antibodies to transcription intermediary factor 1γ/α (TIF1γ/α, p155/140) are frequently found in DM associated with malignancy while anti-melanoma differentiation-associated gene 5 (MDA5; CADM140) are associated with clinically amyopathic DM (CADM) complicated by rapidly progressive ILD. Also, anti-MJ/nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modifier-1 (SUMO-1) activating enzyme (SAE) are recognized as new DM-specific autoantibodies. Addition of these new antibodies to clinical practice in the future will help in making earlier and more accurate diagnoses and better management for patients.
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- 2017
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5. Prevalence and clinical significance of anti-MDA5 antibodies in European patients with polymyositis/dermatomyositis.
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Ceribelli A, Fredi M, Taraborelli M, Cavazzana I, Tincani A, Selmi C, Chan JY, Chan EK, Satoh M, and Franceschini F
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- Adult, Aged, Biomarkers blood, Blotting, Western, Dermatomyositis blood, Dermatomyositis diagnosis, Dermatomyositis epidemiology, Enzyme-Linked Immunosorbent Assay, Female, HeLa Cells, Humans, Immunoprecipitation, Interferon-Induced Helicase, IFIH1, Italy epidemiology, K562 Cells, Male, Middle Aged, Phenotype, Predictive Value of Tests, Prevalence, Retrospective Studies, White People, Autoantibodies blood, DEAD-box RNA Helicases immunology, Dermatomyositis immunology
- Abstract
Objectives: Polymyositis/dermatomyositis (PM/DM) is an autoimmune disease characterised by skin and muscle inflammation, internal organ involvement and serum disease-specific autoantibodies. The recently identified anti-MDA5 (melanoma differentiation-associated gene 5) antibodies are associated with clinically amyopathic DM (CADM), rapidly progressive interstitial lung disease, severe skin manifestations, and poor prognosis. Our objective was to examine the clinical significance of anti-MDA5 antibodies in a cohort of European Caucasian patients with PM/DM, considering that data on anti-MDA5 serology are limited to Asian and US cohorts., Methods: Sera from 76 consecutive adult Italian patients with PM/DM were analysed by immunoprecipitation (IP) of 35S-methionine radiolabelled HeLa and K562 cell extracts, ELISA using recombinant MDA5 protein and IP-Western Blot using rabbit anti-MDA5 antibodies. Clinical associations of anti-MDA5 antibody positive patients were analysed., Results: Anti-MDA5 antibodies were identified in 5/76 (7%) PM/DM cases and all 5 cases were CADM; anti-MDA5 was the second most common autoantibody in DM after anti-MJ/NXP-2, found in 24% of cases. Compared to 29 anti-MDA5 (-) DM, anti-MDA5 (+) patients have more typical DM skin disease (digit pulp/periungual lesions, Gottron's papules, heliotrope rash) (p=ns). Interstitial lung disease was observed in 3/5 anti-MDA5 (+) patients but only 14% of anti-MDA5 (-) cases (p=0.048)., Conclusions: Our study on European patients with PM/DM confirms that anti-MDA5 antibodies are not uncommon. All anti-MDA5 (+) cases are affected by CADM with typical skin disease, while rapidly progressive pulmonary involvement was diagnosed only in one case. Further studies in larger cohorts are necessary to define the clinical significance of anti-MDA5 antibodies in European PM/DM.
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- 2014
6. Rpp25 is a major target of autoantibodies to the Th/To complex as measured by a novel chemiluminescent assay.
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Mahler M, Gascon C, Patel S, Ceribelli A, Fritzler MJ, Swart A, Chan EK, and Satoh M
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- Area Under Curve, Autoantigens immunology, Endoribonucleases immunology, Enzyme-Linked Immunosorbent Assay methods, Humans, Immunoprecipitation, Multienzyme Complexes immunology, ROC Curve, Recombinant Proteins, Sensitivity and Specificity, Autoantibodies immunology, Luminescent Measurements methods, Ribonuclease P immunology, Scleroderma, Systemic immunology
- Abstract
Introduction: Autoantibodies to the Th/To antigen have been described in systemic sclerosis (SSc) and several proteins of the macromolecular Th/To complex have been reported to react with anti-Th/To antibodies. However, anti-Th/To has not been clinically utilized due to unavailability of commercial tests. The objective of the present study is to evaluate the newly developed ELISA and chemiluminescent immunoassay (CLIA) to measure autoantibodies to Rpp25 (a component of the Th/To complex) using immunoprecipitation (IP) as the reference method., Methods: The first cohort consisted of 123 SSc patients including 7 anti-Th/To positive samples confirmed by IP. Additional seven anti-Th/To positive samples from non-SSc patients were also tested. For evaluation of the QUANTA Flash Rpp25 CLIA (research use only), 8 anti-Th/To IP positives, a cohort of 70 unselected SSc patients and sera from various disease controls (n = 357) and random healthy individuals (n = 10) were studied., Results: Anti-Rpp25 antibodies determined by ELISA were found in 11/14 anti-Th/To IP positive but only in 1/156 (0.6%) negative samples resulting in a positive percent agreement of 78.6% (95% confidence interval [CI] 49.2, 95.3%) and a negative percent agreement of 99.4% (95% CI 96.4, 100.0%). To verify the results using a second method, 53 samples were tested by ELISA and CLIA for anti-Rpp25 reactivity and the results were highly correlated (rho = 0.71, 95% CI 0.56, 0.81; P < 0.0001). To define the cutoff of the CLIA, anti-Th/To IP positive and negative sera were tested using the anti-Rpp25 CLIA. At the cutoff selected by receiver operating characteristic (ROC) analysis 8/8 (100.0%) of the anti-Th/To positive sera but only 2/367 (0.5%) of the controls were positive for anti-Rpp25 antibodies. The positive and negative percent agreements were 100.0% (95% CI 63.1, 100.0%) and 99.5% (95% CI 98.0, 99.9%), respectively. In the disease cohorts 2/70 (2.9%) of the SSc patients were positive for anti-Rpp25 antibodies compared to 2/367 (0.5%) of the controls (P = 0.032). ROC analysis showed discrimination between SSc patients and controls with an area under the curve value of 0.732 (95% CI 0.655, 0.809)., Conclusion: Rpp25 is a major target of autoantibodies to the Th/To autoantigen complex. Further studies are needed to evaluate the clinical utility of the new assays.
- Published
- 2013
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7. Differential reactivity to IMPDH2 by anti-rods/rings autoantibodies and unresponsiveness to pegylated interferon-alpha/ribavirin therapy in US and Italian HCV patients.
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Carcamo WC, Ceribelli A, Calise SJ, Krueger C, Liu C, Daves M, Villalta D, Bizzaro N, Satoh M, and Chan EK
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- Antibody Specificity, Autoantibodies blood, Cohort Studies, Hepatitis C epidemiology, Humans, Italy, Recombinant Proteins therapeutic use, Seroepidemiologic Studies, Treatment Outcome, United States, Antiviral Agents therapeutic use, Autoantibodies immunology, Hepatitis C drug therapy, Hepatitis C immunology, IMP Dehydrogenase immunology, Interferon-alpha therapeutic use, Polyethylene Glycols therapeutic use, Ribavirin therapeutic use
- Abstract
Purpose: Autoantibodies to cytoplasmic structures called rods and rings (RR) are primarily specific to patients with hepatitis C virus (HCV) infection treated with pegylated interferon-alpha/ribavirin (IFN/R). Our aim is to examine anti-RR antibodies specificity and correlation with the response to IFN/R therapy in two independent cohorts (US and Italy) of HCV patients., Methods: Sera from the US cohort (n = 47) and the Italian cohort (n = 46) pre-selected for anti-RR antibodies were analyzed by immunofluorescence and radioimmunoprecipitation. The prevalence and titers of anti-RR were analyzed for correlation with the response to IFN/R therapy., Results: In the US cohort, anti-RR antibodies were more frequently non-responders to IFN/R (71 % vs 29 % responders). Titers in responder patients (n = 11) were ≤1:3200, whereas titers in non-responder patients (n = 27) reached 1:819,200 (p = 0.0016). In the Italian cohort, anti-RR titers ranged from 1:200 to >1:819,200 and only relapsers had the highest anti-RR titers. Radioimmunoprecipitation demonstrated that anti-RR autoantibodies were mainly anti-inosine monophosphate dehydrogenase 2 (IMPDH2) - 96 % in the Italian cohort vs. 53 % in the US cohort., Conclusions: In the two cohorts analyzed, the anti-IMPDH2 response as a component of the anti-RR response is much more prominent in the Italian cohort. The reason for the difference between the US and Italian cohorts is unclear but it possibly illustrates the heterogeneity in response and the overall negative correlation between the production of these autoantibodies and response to IFN/R therapy. Patients with high titer anti-RR antibodies are either relapsers (Italian) or non-responders/relapsers (US).
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- 2013
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8. Autoantibodies to Argonaute 2 (Su antigen).
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Satoh M, Chan JY, Ceribelli A, Vazquez del-Mercado M, and Chan EK
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- Animals, Antigen-Antibody Complex metabolism, Argonaute Proteins genetics, Argonaute Proteins metabolism, Autoantibodies genetics, Autoantibodies metabolism, Autoimmunity, Fluorescent Antibody Technique, Humans, Immunodiffusion, Mice, MicroRNAs genetics, MicroRNAs immunology, Protein Binding, Proteins genetics, Proteins metabolism, RNA Interference immunology, Rheumatic Diseases genetics, Rheumatic Diseases metabolism, Antigen-Antibody Complex genetics, Argonaute Proteins immunology, Autoantibodies immunology, MicroRNAs metabolism, Proteins immunology, Rheumatic Diseases immunology
- Abstract
Like many other classical autoantibodies in systemic rheumatic diseases, anti-Su antibodies were originally defined by the double immunodiffusion assay in the early 80s. However, despite its high prevalence, only a few reports on anti-Su were published in the following years and the progress in characterizing the target antigens and clinical significance was slow, probably due to its inconsistent or poor reactivity in other standard immunoassays. In 2006 the target antigen was identified as the microRNA (miRNA)-binding protein Argonaute 2 (Ago2). Ago2 is a key component of the RNA-induced silencing complex enriched in cytoplasmic foci called GW bodies. Due to preferential reactivity of human autoantibodies with native antigens, immunoprecipitation is the only method to reliably detect anti-Su/Ago2 antibodies. Anti-Su/Ago2 does not appear to have disease specificity since it is found in 10-20% of patients with various rheumatic diseases, including systemic lupus erythematosus, scleroderma, polymyositis/dermatomyositis, and Sjögren's syndrome, as well as apparently healthy individuals at lower prevalence. The clinical significance and the mechanism of production of anti-Su/Ago2 remains to be clarified.
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- 2013
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9. Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis.
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Ceribelli A, Fredi M, Taraborelli M, Cavazzana I, Franceschini F, Quinzanini M, Tincani A, Ross SJ, Chan JY, Pauley BA, Chan EK, and Satoh M
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- Adult, Aged, Biomarkers metabolism, Cohort Studies, Dermatomyositis diagnosis, Female, Humans, Italy epidemiology, K562 Cells, Male, Middle Aged, Adenosine Triphosphatases immunology, Antibody Specificity, Autoantibodies biosynthesis, DNA-Binding Proteins immunology, Dermatomyositis epidemiology, Dermatomyositis immunology
- Abstract
Introduction: Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied., Methods: Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts., Results: Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent nuclear dots staining, despite PML localization of NXP-2/MORC3., Conclusions: Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
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- 2012
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10. Autoantibodies to transcription intermediary factor TIF1β associated with dermatomyositis.
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Satoh M, Chan JY, Ross SJ, Li Y, Yamasaki Y, Yamada H, Vazquez-del Mercado M, Petri MH, Jara LJ, Saavedra MA, Cruz-Reyes C, Sobel ES, Reeves WH, Ceribelli A, and Chan EK
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- Amino Acid Sequence, Biomarkers blood, Female, Humans, K562 Cells, Molecular Sequence Data, Muscular Diseases diagnosis, Muscular Diseases immunology, Registries, Tripartite Motif-Containing Protein 28, Autoantibodies biosynthesis, Dermatomyositis diagnosis, Dermatomyositis immunology, Repressor Proteins immunology
- Abstract
Introduction: Myositis specific autoantibodies are associated with unique clinical subsets and are useful biomarkers in polymyositis/dermatomyositis (PM/DM). A 120 kD protein recognized by certain patients with DM was identified and clinical features of patients with this specificity were characterized., Methods: The 120 kD protein recognized by a prototype serum was purified and identified by mass spectrometry and immunological methods. Autoantibody to this 120 kD protein was screened in sera from 2,356 patients with various diagnoses from four countries, including 254 PM/DM, by immunoprecipitation of 35S-methionine labeled K562 cell extracts. Clinical information of patients with this specificity was collected., Results: The 120 kD protein, which exactly comigrated with PL-12, was identified as transcription intermediary factor TIF1β (TRIM28) by mass spectrometry and validated by immunoassays. By immunofluorescence, anti-TIF1β positivity showed a fine-speckled nuclear staining pattern. Four cases of anti-TIF1β were identified; all are women, one each in a Japanese, African American, Caucasian, and Mexican individual. Three had a diagnosis of DM and one case was classified as having an undifferentiated connective tissue disease with an elevated CPK but without significant muscle symptoms. This individual also had a history of colon cancer, cervical squamous metaplasia and fibroid tumors of the uterus. Myopathy was mild in all cases and resolved without treatment in one case. The anti-TIF1β specificity was not found in other conditions., Conclusions: Anti-TIF1β is a new DM autoantibody associated with a mild form of myopathy. Whether it has an association with malignancy, as in the case of anti-TIF1γ, or other unique features will need to be evaluated in future studies.
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- 2012
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11. Role of environmental factors in autoantibody production - importance of a detailed analysis in a small cohort.
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Satoh M, Ceribelli A, and Chan EK
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- Female, Humans, Male, Autoantibodies immunology, DEAD-box RNA Helicases immunology, Dermatomyositis immunology
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- 2012
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12. International cohort study of 73 anti-Ku-positive patients: association of p70/p80 anti-Ku antibodies with joint/bone features and differentiation of disease populations by using principal-components analysis.
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Lakota K, Thallinger GG, Sodin-Semrl S, Rozman B, Ambrozic A, Tomsic M, Praprotnik S, Cucnik S, Mrak-Poljsak K, Ceribelli A, Cavazzana I, Franceschini F, Vencovsky J, Czirják L, Varjú C, Steiner G, Aringer M, Stamenkovic B, Distler O, Matucci-Cerinic M, and Kveder T
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- Adolescent, Adult, Aged, Aged, 80 and over, Arthritis, Rheumatoid blood, Arthritis, Rheumatoid immunology, Autoantibodies blood, Bone Diseases blood, Cohort Studies, Counterimmunoelectrophoresis, Europe, Female, Humans, Immunoassay, Immunoblotting, Joint Diseases blood, Ku Autoantigen, Lupus Erythematosus, Systemic blood, Lupus Erythematosus, Systemic immunology, Male, Middle Aged, Polymyositis blood, Polymyositis immunology, Scleroderma, Systemic blood, Scleroderma, Systemic immunology, Sjogren's Syndrome blood, Sjogren's Syndrome immunology, Young Adult, Antigens, Nuclear immunology, Autoantibodies immunology, Bone Diseases immunology, DNA-Binding Proteins immunology, Joint Diseases immunology, Principal Component Analysis
- Abstract
Introduction: An international cohort study of 73 anti-Ku-positive patients with different connective tissue diseases was conducted to differentiate the anti-Ku-positive populations of patients based on their autoantibody profile and clinical signs/symptoms and to establish possible correlations between antibodies against Ku p70 and Ku p80 with autoimmune diseases., Methods: Sera of anti-Ku-positive patients were collected from six European centers and were all secondarily tested (in the reference center); 73 were confirmed as positive. Anti-Ku antibodies were detected with counter-immunoelectrophoresis (CIE), line immunoassay (LIA), and immunoblot analyses. All clinical and laboratory data were follow-up cumulative data, except for anti-Ku antibodies. Statistical analyses were performed by using R (V 2.12.1). The Fisher Exact test was used to evaluate the association between anti-Ku antibodies and diagnosis, gender, clinical signs, and other observed antibodies. The P values were adjusted for multiple testing. Separation of disease populations based on the presence of antibodies and clinical signs was investigated by principal-components analysis, which was performed by using thr// R's prcomp function with standard parameters., Results: A 16% higher prevalence of anti-Ku p70 was found over anti-Ku p80 antibodies. In 41 (57%) patients, a combination of both was detected. Five (7%) patients, who were CIE and/or LIA anti-Ku positive, were negative for both subsets, as detected with the immunoblot; 31% of the patients had undifferentiated connective tissue disease (UCTD); 29% had systemic sclerosis (SSc); 18% had systemic lupus erythematosus (SLE); 11% had rheumatoid arthritis; 7% had polymyositis; and 3% had Sjögren syndrome., Conclusions: A significant positive association was found between female patients with anti-Ku p70 and joint/bone features, and a significant negative association was found between female patients with anti-Ku p80 only and joint/bone features (P = 0.05, respectively). By using the first and the third components of the principal-component analysis (PCA) with 29 parameters evaluated, we observed that the anti-Ku-positive population of UCTD patients had overlapping parameters, especially with SLE, as opposed to SSc, which could be helpful in delineating UCTD patients.
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- 2012
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13. Primary biliary cirrhosis-related autoantibodies in a large cohort of italian patients with systemic sclerosis.
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Cavazzana I, Ceribelli A, Taraborelli M, Fredi M, Norman G, Tincani A, Satoh M, and Franceschini F
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- Adult, Aged, Antigens, Nuclear immunology, Autoantigens immunology, Female, Humans, Italy, Liver Cirrhosis, Biliary immunology, Male, Middle Aged, Nuclear Pore Complex Proteins immunology, Predictive Value of Tests, Recombinant Fusion Proteins immunology, Sensitivity and Specificity, Autoantibodies immunology, Liver Cirrhosis, Biliary diagnosis, Scleroderma, Systemic immunology
- Abstract
Objective: To analyze the prevalence, associations, and fine specificity of autoantibodies to primary biliary cirrhosis (PBC)-associated antigens (MIT3, Sp100, and gp210) in a cohort of Italian patients with systemic sclerosis (SSc)., Methods: Sera samples from 201 patients with SSc were tested for antibodies to MIT3, gp210, and Sp100 by ELISA (the PBC screen). Anti-MIT3-positive sera were studied for IgG or IgA isotypes. All sera were analyzed by indirect immunofluorescence on HEp-2 cells and on rodent kidney/stomach/liver tissue sections in order to detect antinuclear and antimitochondrial antibodies (AMA). SSc was selected by American College of Rheumatology criteria and classified based on LeRoy's criteria., Results: Forty-three (21.4%) sera samples were positive for PBC screen antibodies. Anti-MIT3 antibodies were detected in 36 samples, anti-Sp100 in 5, and anti-gp210 in 1 sample. The other 3 PBC screen-positive samples showed no specificity for the single antigens. PBC screen-positive patients more frequently showed a limited cutaneous SSc subtype (p = 0.04), anticentromere antibodies (ACA; p = 0.0013), elevated alkaline phosphatase (ALP) (p < 0.0001), PBC (p = 0.002), and AMA (p = 0.008). Teleangiectasia and calcinosis were less frequent in this group of patients. IgG+IgA anti-MIT3-positive patients had higher prevalence of AMA (p = 0.0035), diagnosis of PBC (p = 0.014), and increased ALP (p = 0.039), all considered biochemical markers of severe liver disease., Conclusion: PBC screen antibodies were detected in 20% of patients with SSc, strongly associated with ACA. ACA+/PBC screen+ patients had higher risk of developing PBC or elevation of ALP.
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- 2011
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14. Atypical clinical presentation of a subset of patients with anti-RNA polymerase III--non-scleroderma cases associated with dominant RNA polymerase I reactivity and nucleolar staining.
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Ceribelli A, Krzyszczak ME, Li Y, Ross SJ, Chan JY, Chan EK, Burlingame RW, Webb TT, Bubb MR, Sobel ES, Reeves WH, and Satoh M
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- Adult, Aged, Autoantibodies immunology, Autoantigens immunology, Cell Nucleolus immunology, Cell Nucleolus metabolism, Enzyme-Linked Immunosorbent Assay, Female, Fluorescent Antibody Technique, Humans, Middle Aged, Radioimmunoprecipitation Assay, Raynaud Disease diagnosis, Raynaud Disease metabolism, Scleroderma, Systemic diagnosis, Scleroderma, Systemic metabolism, Autoantibodies blood, RNA Polymerase I immunology, RNA Polymerase III immunology, Raynaud Disease immunology, Scleroderma, Systemic immunology
- Abstract
Introduction: Anti-RNA polymerase III (RNAP III) antibodies are highly specific markers of scleroderma (systemic sclerosis, SSc) and associated with a rapidly progressing subset of SSc. The clinical presentation of anti-RNAP III positive patients, onset of Raynaud's phenomenon (RP) and SSc in unselected patients in a rheumatology clinic were evaluated., Methods: Autoantibodies in sera from 1,966 unselected patients (including 434 systemic lupus erythematosus (SLE), 119 SSc, 85 polymyositis/dermatomyositis (PM/DM)) in a rheumatology clinic were screened by radioimmunoprecipitation. Anti-RNAP III positive sera were also tested by immunofluorescence antinuclear antibodies and anti-RNAP III ELISA. Medical records of anti-RNAP III positive patients were reviewed., Results: Among 21 anti-RNAP III positive patients, 16 met the American College of Rheumatology (ACR) SSc criteria at the initial visit but 5 did not; diagnoses were vasculitis, early polyarthritis, renal failure with RP, interstitial lung disease, and Sjögren's syndrome. The first two patients developed rapidly progressive diffuse SSc. An additional case presented with diffuse scleroderma without RP and RP developed two years later. Anti-RNAP III antibodies in these 6 cases of atypical clinical presentation were compared with those in 15 cases of typical (SSc with RP) cases. Anti-RNAP III levels by ELISA were lower in the former group (P = 0.04 by Mann-Whitney test) and 3 of 6 were negative versus only 1 of 15 negative in the latter (P < 0.05 by Fisher's exact test). Three cases of non-SSc anti-RNAP III positive patients had predominant reactivity with RNAP I with weak RNAP III reactivity and had a strong nucleolar staining. Three anti-RNAP III patients, who did not have RP at the initial visit, developed RP months later. Scleroderma developed prior to RP in 5 out of 16 (31%) in the anti-RNAP III group, but this was rare in patients with other autoantibodies. The interval between the onset of RP to scleroderma was short in anti-RNAP III positive patients., Conclusions: Anti-RNAP III antibodies are highly specific for SSc; however, a subset of anti-RNAP III positive patients do not present as typical SSc. The interval between RP and scleroderma in this group is short, and 31% of patients developed scleroderma prior to RP in this group. Anti-RNAP III positive patients may not present as typical SSc and detecting anti-RNAP III may have predictive value.
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- 2011
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15. Autoantibodies to survival of motor neuron complex in patients with polymyositis: immunoprecipitation of D, E, F, and G proteins without other components of small nuclear ribonucleoproteins.
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Satoh M, Chan JY, Ross SJ, Ceribelli A, Cavazzana I, Franceschini F, Li Y, Reeves WH, Sobel ES, and Chan EK
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- Adult, Autoimmune Diseases immunology, Blotting, Western, Female, Fluorescent Antibody Technique, Humans, Immunoprecipitation, Lupus Erythematosus, Systemic immunology, Male, Middle Aged, Scleroderma, Systemic immunology, Autoantibodies immunology, Polymyositis immunology, Ribonucleoproteins, Small Nuclear immunology, SMN Complex Proteins immunology
- Abstract
Objective: Autoantibodies in the systemic rheumatic diseases are clinically useful biomarkers of the diagnosis or of certain clinical characteristics. An unusual pattern of immunoprecipitation, in which the D, E, F, and G proteins of small nuclear RNPs (snRNP) but without other components of the snRNP, was noticed at the autoantibody screening. The purpose of this study was to examine the target antigens and clinical manifestations associated with this specificity., Methods: Autoantibodies in sera from 1,966 American patients (including 434 with systemic lupus erythematosus, 121 with scleroderma, 86 with polymyositis/dermatomyositis [PM/DM]) and 248 Italian patients with autoimmune diseases were screened by immunoprecipitation of (35) S-methionine-labeled cell extracts. Sera with which D, E, F, and G proteins of snRNP was immunoprecipitated, but without the other snRNP proteins, were further examined by analysis of RNA components by immunoprecipitation (silver staining), Western blotting using survival of motor neuron (SMN) complex, and immunofluorescence., Results: Three sera that immunoprecipitated D, E, F, and G proteins without other components (U1-70K, A, B'/B, C) of the snRNP were found. Four additional proteins (130 kd, 120 kd, 38 kd, and 33 kd) were also commonly immunoprecipitated. The target antigen was identified as SMN complex (Gemin 3, Gemin 4, SMN, and Gemin 2, respectively), which plays a critical role in the assembly of snRNP. In immunofluorescence analyses, all 3 sera showed nuclear dots (Cajal bodies) and cytoplasmic staining. Only 1 serum was weakly positive on Western blotting of SMN, suggesting that these sera mainly recognize native molecule or quaternary structure. All 3 patients were white women with PM, an interesting finding, since deletion or mutation of SMN is known to cause spinal muscular atrophy., Conclusion: SMN complex was identified as a new Cajal body autoantigen recognized by sera from white patients with PM. The biologic and clinical significance of anti-SMN autoantibodies will need to be clarified., (Copyright © 2011 by the American College of Rheumatology.)
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- 2011
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16. Anti-argonaute2 (Ago2/Su) and -Ro antibodies identified by immunoprecipitation in primary anti-phospholipid syndrome (PAPS).
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Ceribelli A, Tincani A, Cavazzana I, Franceschini F, Cattaneo R, Pauley BA, Chan JY, Chan EK, and Satoh M
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- Adult, Animals, Antibodies, Antiphospholipid blood, Antibodies, Antiphospholipid immunology, Antibody Specificity, Antiphospholipid Syndrome diagnosis, Argonaute Proteins, Autoantibodies immunology, Dogs, Enzyme-Linked Immunosorbent Assay, Female, Humans, Lupus Erythematosus, Systemic immunology, Male, Mice, Middle Aged, Rabbits, Young Adult, Antiphospholipid Syndrome immunology, Autoantibodies blood, Eukaryotic Initiation Factor-2 immunology, Immunoprecipitation methods, Ribonucleoproteins immunology
- Abstract
Objectives: Primary anti-phospholipid syndrome (PAPS) is an autoimmune condition defined by anti-phospholipid antibodies (aPL) and thrombotic or obstetric events. Some PAPS can evolve into systemic lupus erythematosus (SLE) during follow-up. Few studies systematically examined lupus autoantibodies and their clinical significance in PAPS. The aim of our study is to analyze the clinical and laboratory correlations with lupus-related autoantibodies, detected by immunoprecipitation (IP), a technique not yet systematically applied to investigate autoantibodies in this condition., Methods: Sera from 52 PAPS patients were screened by indirect immunofluorescence (IIF) antinuclear antibodies (ANA), IP of ³⁵S-labeled K562 cell extract, and ELISA [anti-Argonaute2 (Ago2, Su), 60kRo, 52kRo, La, dsDNA)]. Anti-Ago2/Su positive sera were also tested for anti-GW bodies (GWBs) by IIF double staining, using rabbit anti-Rck/p54 serum., Results: First, 56% of PAPS patients (29/52) were ANA positive, mainly with speckled pattern. Anti-Ago2/Su antibodies were found in 13% (7/52), anti-Ro/SSA in 10% (5/52), anti-La in one case. The clinical profile of patients did not seem to be related to the presence of these antibody specificities. However, levels of IgG anti-β2 glycoprotein I antibodies were lower in anti-Ago2/Su positive patients (p = 0.02). None of anti-Ago2/Su or -Ro patients developed SLE during a 2-year follow-up. Ago2 is a key component of GWBs, however, only 1/7 anti-Ago2/Su serum showed a typical cytoplasmic GWBs staining., Conclusions: Anti-Ago2/Su and -Ro antibodies are the two autoantibodies detected by IP in our PAPS cohort. Clarifying why Ago2/Su and Ro are specific targets of autoimmunity may help to understand the mechanisms of autoantibody production.
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- 2011
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17. Anti-Th/To are common antinucleolar autoantibodies in Italian patients with scleroderma.
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Ceribelli A, Cavazzana I, Franceschini F, Airò P, Tincani A, Cattaneo R, Pauley BA, Chan EK, and Satoh M
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- Adult, Aged, Antibodies, Antinuclear immunology, Autoantibodies immunology, Female, Humans, Italy, Male, Middle Aged, Antibodies, Antinuclear blood, Autoantibodies blood, Cell Nucleolus immunology, Scleroderma, Systemic blood, Scleroderma, Systemic immunology
- Abstract
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)., Methods: Sera from 216 consecutive patients with SSc were tested for ACA (by indirect immunofluorescence), antitopoisomerase I (topo I; by counterimmunoelectrophoresis), and anti-RNA polymerase III (RNAPIII; by ELISA). Forty-one sera negative for these specificities were tested by IP analysis of proteins ((35)S-methionine labeled K562 cell extract) and RNA (silver staining)., Results: Among 216 SSc patients analyzed, anti-topo I, ACA, and anti-RNAPIII were detected in 38% (81/216), 31% (67/216) and 7% (15/216), respectively. Among 41 sera negative for ACA, anti-topo I, and anti-RNAPIII and which were tested by IP, 14 were nucleolar stain-positive. Eight out of 14 (57%) showed anti-Th/To reactivity, but no anti-U3RNP was found. In comparison with ACA-positive patients, anti-Th/To-positive patients were younger (p = 0.0046) and more commonly were male (p = 0.0006). All 8 anti-Th/To-positive and all but one ACA-positive patients had lcSSc. Interstitial lung disease (ILD) and pericarditis were more frequent in anti-Th/To-positive patients., Conclusion: Anti-Th/To are common in antinucleolar antibody-positive Italian patients with SSc. Anti-Th/To and ACA patients had lcSSc, with excellent prognosis. The anti-Th/To group had frequent pericarditis and ILD, although impairment of pulmonary function appeared mild.
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- 2010
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18. Anti-RNA polymerase III antibodies as a risk marker for early gastric antral vascular ectasia (GAVE) in systemic sclerosis.
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Ceribelli A, Cavazzana I, Airò P, and Franceschini F
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- Biomarkers blood, Disease Progression, Humans, Retrospective Studies, Risk Factors, Autoantibodies blood, Gastric Antral Vascular Ectasia blood, Gastric Antral Vascular Ectasia etiology, Gastric Antral Vascular Ectasia immunology, RNA Polymerase III immunology, Scleroderma, Systemic blood, Scleroderma, Systemic complications, Scleroderma, Systemic immunology
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- 2010
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19. Anti-RNA polymerase III antibodies: a marker of systemic sclerosis with rapid onset and skin thickening progression.
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Cavazzana I, Ceribelli A, Airo' P, Zingarelli S, Tincani A, and Franceschini F
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- Antibody Specificity, Autoantibodies immunology, Biomarkers blood, Disease Progression, Female, Humans, Male, Middle Aged, Scleroderma, Systemic blood, Skin immunology, Autoantibodies blood, RNA Polymerase III immunology, Scleroderma, Systemic immunology, Scleroderma, Systemic pathology, Skin pathology
- Abstract
Anti-RNA polymerase III antibodies (ARA) are a specific marker for Systemic Sclerosis (SSc), associated to severe disease with major organ and diffuse cutaneous involvement. In our series, ARA were found in 19 of 216 sera, in 15 cases as isolated antibodies' specificity, with a statistically negative association with other SSc-specific autoantibodies (p: 0.00003). The prevalence of ARA among 73 anticentromere and anti-topoisomerase I (topo I) negative sera, was 20.5%. Patients with isolated ARA had more rapid disease onset, defined as the interval from the appearance of Raynaud's phenomenon to the first symptom other than Raynaud's, than patients with isolated anti-topo I antibodies (median: 2 months vs 13 months; p: 0.0013). A rapid onset of SSc (within 6 months from Raynaud's phenomenon onset) was found in all patients with isolated ARA and only in 34% of those with anti-topo I (p<0.00001). Moreover, the skin thickening in the first months after SSc onset was faster in the ARA group (p<0.0001). Nevertheless, the rates of internal organ involvement and of survival rates were similar between the two groups. Our experience therefore suggests that ARA are a marker of very rapid onset of disease and skin thickening progression in SSc.
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- 2009
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20. An Italian Multicenter Study on Anti-NXP2 Antibodies: Clinical and Serological Associations
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Fredi, Micaela, Cavazzana, Ilaria, Ceribelli, Angela, Cavagna, Lorenzo, Barsotti, Simone, Bartoloni, Elena, Benucci, Maurizio, De Stefano, Ludovico, Doria, Andrea, Emmi, Giacomo, Fabris, Martina, Fornaro, Marco, Furini, Federica, Giudizi, Maria Grazia, Govoni, Marcello, Ghirardello, Anna, Iaccarino, Luca, Iannone, Fiorenzo, Infantino, Maria, Isailovic, Natasa, Lazzaroni, Maria Grazia, Manfredi, Mariangela, Mathieu, Alessandro, Marasco, Emiliano, Migliorini, Paola, Montecucco, Carlomaurizio, Palterer, Boaz, Parronchi, Paola, Piga, Matteo, Pratesi, Federico, Riccieri, Valeria, Selmi, Carlo, Tampoia, Marilina, Tripoli, Alessandra, Zanframundo, Giovanni, Radice, Antonella, Gerli, Roberto, and Franceschini, Franco
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- 2022
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21. Autoimmune Polyendocrine Syndromes
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Tincani, Angela, Ceribelli, Angela, Cavazzana, Ilaria, Franceschini, Franco, Sulli, Alberto, Cutolo, Maurizio, Shoenfeld, Yehuda, editor, Cervera, Ricard, editor, and Gershwin, M. Eric, editor
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- 2008
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22. Common Pathways of Autoimmune Inflammatory Myopathies and Genetic Neuromuscular Disorders
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Satoh, Minoru, Ceribelli, Angela, and Chan, Edward K. L.
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- 2012
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23. Gender and ethnicity differences in the prevalence of scleroderma-related autoantibodies
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Krzyszczak, Malgorzata E., Li, Yi, Ross, Steven J., Ceribelli, Angela, Chan, Edward K. L., Bubb, Michael R., Sobel, Eric S., Reeves, Westley H., and Satoh, Minoru
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- 2011
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24. Antigen Reactivity and Clinical Significance of Autoantibodies Directed Against the Pyruvate Dehydrogenase Antigen Complex in Patients With Connective Tissue Disease.
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Ceribelli, Angela, Isailovic, Natasa, Gorlino, Carolina, Assandri, Roberto, Vecellio, Matteo, De Santis, Maria, Satoh, Minoru, and Selmi, Carlo
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PYRUVATE dehydrogenase complex ,CONNECTIVE tissue diseases ,AUTOANTIBODIES ,AUTOIMMUNE diseases ,ANTIGENS ,SYSTEMIC scleroderma - Abstract
Introduction: Antimitochondrial antibodies (AMAs) are the hallmark of primary biliary cholangitis (PBC) but can be identified also in patients with connective tissue disease, namely, systemic sclerosis (SSc). Protein immunoprecipitation (IP) and IP-Western blot (WB) can be used to confirm AMA positivity directed at the pyruvate dehydrogenase complex (PDC) subunits E1α, E1β, E2/E3, and E3BP in patients showing a cytoplasmic reticular pattern at indirect immunofluorescence when performed in a screening setting before the onset of overt cholestasis in rheumatic patients. Patients and Methods: We studied sera from 285 patients affected by connective tissue disease [SSc, n = 144; dermato/polymyositis (DM/PM), n = 56; and undifferentiated connective tissue disease (UCTD), n = 85] by indirect immunofluorescence (IIF), protein-IP, and IP-WB to identify specific PDC subunits recognized by AMA. Results: Twenty percent (57/285) of sera from patients with connective tissue disease had a cytoplasmic reticular pattern at IIF, and in 77% (44/57, including 20 SSc, 12 PM/DM, and 12 UCTD) of these, we detected different titers of autoantibodies against the PDC subunits, specifically against PDC-E2. Among these sera, 4 (9%) tested positive for anti-E1α, 15 (34%) for anti-E1β, and 16 (36%) for anti-E3BP. Four of the 20 AMA-positive SSc cases (20%) had been already diagnosed with PBC, and all were positive for autoantibodies against the subunits PDC-E2, E3, and E3BP. Conclusions: Using IIF and IP, we confirm that autoantibodies against the PDC components are detected in rheumatic patients with PBC or without liver dysfunction. In view of the strong predictive value of AMA for PBC, a strict follow-up of these latter patients is warranted for an early diagnosis of the disease. [ABSTRACT FROM AUTHOR]
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- 2022
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25. Anti-cyclic citrullinated peptide antibodies in systemic lupus erythematosus patients with articular involvement: a predictive marker for erosive disease?
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Taraborelli, M, Inverardi, F, FREDI - NON UTILIZZARE, Micaela, Ceribelli, Angela, Cavazzana, Ilaria, Tincani, Angela, Franceschini, F., and Fredi, Micaela
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Adult ,Male ,musculoskeletal diseases ,lcsh:Internal medicine ,Arthritis ,lcsh:Medicine ,Systemic Lupus Erythematosus, anti-cyclic citrullinated peptide antibodies, arthtritis ,Autoantigens ,Peptides, Cyclic ,Rheumatology ,Antibody Specificity ,Predictive Value of Tests ,immune system diseases ,Hand Deformities, Acquired ,Medicine ,Rheumatoid factor ,Humans ,Lupus Erythematosus, Systemic ,skin and connective tissue diseases ,lcsh:RC31-1245 ,Autoantibodies ,Retrospective Studies ,Lupus erythematosus ,Predictive marker ,Systemic lupus erythematosus ,biology ,business.industry ,Foot Deformities, Acquired ,C-reactive protein ,lcsh:R ,medicine.disease ,Prognosis ,Predictive value of tests ,Immunology ,biology.protein ,Female ,Joints ,Antibody ,business - Abstract
A small number of systemic lupus erythematosus (SLE) patients develop an erosive disease. Some studies have suggested an association between anti-cyclic citrullinated (anti-CCP) antibodies and this pattern of arthritis, but their exact significance in SLE patients remains unclear. The aim of this study was to evaluate the prevalence of anti-CCP antibodies in SLE patients with different subsets of articular disease. Among 521 SLE patients followed in this center from 1976 to 2011, those with articular involvement (n=298) were selected to take part in the study. We searched for anti-CCP2 IgG antibodies in 198 patients using a commercial enzyme linked immunosorbent assay (Immunoscan RA, Eurodiagnostica). In 174 patients the results for rheumatoid factor (RF) by nephelometry were retrospectively collected. C reactive protein (CRP) was obtained from clinical records. Patients were classified into 3 groups: erosive, non-erosive deforming, non-erosive non-deforming arthritis. Results of the different tests were compared among the groups. Plt;0.05 was considered statistically significant. Anti-CCP antibodies were significantly associated with erosive disease. We also found that RF positivity and increased CRP were more frequent in erosive arthritis and erosive or non-deforming arthritis, respectively, than in non-erosive non-deforming arthritis. This study supports the evidence that anti-CCP antibodies could be a useful marker of erosive disease in SLE patients. Increase in RF and CRP could be an additional means of identifying lupus patients with arthritis at risk of a worse prognosis.
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- 2012
26. Testing for myositis specific autoantibodies: Comparison between line blot and immunoprecipitation assays in 57 myositis sera
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Cavazzana, Ilaria, Fredi, Micaela, Ceribelli, Angela, Mordenti, Cristina, Ferrari, Fabio, Carabellese, Nice, Tincani, Angela, Satoh, Minoru, and Franceschini, Franco
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0301 basic medicine ,Adult ,Male ,Anti-Jo1 ,Anti-synthetase syndrome ,Autoantibodies ,Immunoprecipitation ,Line blot ,Myositis ,Immunology ,Immunology and Allergy ,Pathology ,medicine.medical_specialty ,Interferon-Induced Helicase, IFIH1 ,Polymyositis ,Sensitivity and Specificity ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Retrospective Studies ,030203 arthritis & rheumatology ,Adenosine Triphosphatases ,Immunoassay ,medicine.diagnostic_test ,biology ,business.industry ,Autoantibody ,Gold standard (test) ,Dermatomyositis ,Middle Aged ,medicine.disease ,Molecular biology ,Blot ,DNA-Binding Proteins ,030104 developmental biology ,Antibodies, Antinuclear ,biology.protein ,Female ,Antibody ,business ,K562 Cells ,Transcription Factors - Abstract
Objective To analyze the performance of a line blot assay for the identification of autoantibodies in sera of patients affected by myositis, compared with immunoprecipitation (IP) as gold standard. Methods 66 sera of patients with myositis (23 polymyositis, 8 anti-synthetase syndromes, 29 dermatomyositis and 6 overlap syndromes) were tested by commercial LB (Euroimmun, Lubeck, Germany); 57 sera were analyzed also by IP of K562 cell extract radiolabeled with 35 S-methionine. Inter-rater agreement was calculated with Cohen's k coefficient. Results Myositis-specific antibodies (MSA) were detected in 36/57 sera (63%) by IP and in 39/66 sera (59%) by LB. The most frequent MSA found by LB were anti-Jo1 and anti-Mi2 found in 15% (10/66) of sera, followed by anti-NXP2 and anti-SRP detected in 106% (7/66) of sera. Anti-TIF1gamma and anti-MDA5 were found in 6 (9%) and 5 sera (7.6%), respectively. A good agreement between methods was found only for anti-TIF1γ, anti-MDA5 and anti-NXP-2 antibodies, while a moderate agreement was estimated for anti-Mi2 and anti-EJ. By contrast, a high discordance rate for the detection of anti-Jo1 antibodies was evident (k: 0.3). Multiple positivity for MSA were found in 11/66 (17%) by LB and 0/57 by IP (p: 0001). Comparing the clinical features of these 11 sera, we found total discrepancies between assays in 3 sera (27.3%), a relative discrepancy due to the occurrence of one discordant autoantibody (not confirmed by IP) in 5 cases (45.5%) and a total discrepancy between LB and IP results, but with a relative concordance with clinical features were found in other 3 sera (27.3%). The semiquantitative results do not support the interpretation of the data. Conclusions The use of LB assay allowed the detection of new MSA, such as anti-MDA5, anti-MJ and anti-TIF1gamma antibodies, previously not found with routine methods. However, the high prevalence of multiple positivities and the high discondant rate of anti-Jo1 antibodies could create some misinterpretation of the results from the clinical point of view. These data should be confirmed by enlarging the number of myositis cases.
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- 2016
27. Anti-RNA polymerase III antibodies: A marker of systemic sclerosis with rapid onset and skin thickening progression
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Ilaria, Cavazzana, Angela, Ceribelli, Ceribelli, Angela, Paolo, Airo', Airo', Paolo, Stefania, Zingarelli, Zingarelli, Stefania, Angela, Tincani, Tincani, Angela, Franco, Franceschini, and Franceschini, Franco
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Anti-nuclear antibody ,Immunology ,Rapid systemic sclerosis onset ,Systemic Sclerosis ,Disease ,Scleroderma ,RNA polymerase III ,Antibody Specificity ,medicine ,Humans ,Immunology and Allergy ,skin and connective tissue diseases ,Autoantibodies ,Skin ,Scleroderma, Systemic ,integumentary system ,biology ,Anti-RNA polymerase III ,Skin thickening ,business.industry ,Systemic ,Autoantibody ,RNA Polymerase III ,Middle Aged ,Anti-nuclear antibodies ,medicine.disease ,Raynaud's phenomenon ,Skin thickening progression ,Rapid onset ,Disease Progression ,biology.protein ,Female ,Biomarkers ,Antibody ,business - Abstract
Anti-RNA polymerase III antibodies (ARA) are a specific marker for Systemic Sclerosis (SSc), associated to severe disease with major organ and diffuse cutaneous involvement. In our series, ARA were found in 19 of 216 sera, in 15 cases as isolated antibodies' specificity, with a statistically negative association with other SSc-specific autoantibodies (p: 0.00003). The prevalence of ARA among 73 anticentromere and anti-topoisomerase I (topo I) negative sera, was 20.5%. Patients with isolated ARA had more rapid disease onset, defined as the interval from the appearance of Raynaud's phenomenon to the first symptom other than Raynaud's, than patients with isolated anti-topo I antibodies (median: 2 months vs 13 months; p: 0.0013). A rapid onset of SSc (within 6 months from Raynaud's phenomenon onset) was found in all patients with isolated ARA and only in 34% of those with anti-topo I (p
- Published
- 2009
28. Personalized medicine in rheumatology: the paradigm of serum autoantibodies.
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Sirotti, Silvia, Generali, Elena, Ceribelli, Angela, Isailovic, Natasa, Santis, Maria, and Selmi, Carlo
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RHEUMATOLOGY ,INDIVIDUALIZED medicine ,AUTOANTIBODIES ,ANTINUCLEAR factors ,AUTOIMMUNE diseases - Abstract
The sequencing of the human genome is now well recognized as the starting point of personalized medicine. Nonetheless, everyone is unique and can develop different phenotypes of the same disease, despite identical genotypes, as well illustrated by discordant monozygotic twins. To recognize these differences, one of the easiest and most familiar examples of biomarkers capable of identifying and predicting the outcome of patients is represented by serum autoantibodies. In this review, we will describe the concept of personalized medicine and discuss the predictive, prognostic and preventive role of antinuclear antibodies (ANA), anti-citrullinated peptide antibodies (ACPA), rare autoantibodies and anti-drug antibodies (ADA), to evaluate how these can help to identify different disease immune phenotypes and to choose the best option for treating and monitoring rheumatic patients in everyday practice. The importance of ANA resides in the prediction of clinical manifestations in systemic sclerosis and systemic lupus erythematosus and their association with malignancies. ACPA have a predictive role in rheumatoid arthritis, they are associated with the development of a more aggressive disease, extra-articular manifestations and premature mortality in RA patients; moreover, they are capable of predicting therapeutic response. Rare autoantibodies are associated with different disease manifestations and also with a greater incidence of cancer. The determination of ADA levels may be useful in patients where the clinical efficacy of TNF-α inhibitor has dropped, for the assessment of a right management. The resulting scenario supports serum autoantibodies as the cornerstone of personalized medicine in autoimmune diseases. [ABSTRACT FROM AUTHOR]
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- 2017
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29. MicroRNAs in rheumatoid arthritis
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Ceribelli, Angela, Nahid, Md A., Satoh, Minoru, and Chan, Edward K.L.
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MICRORNA , *RHEUMATOID arthritis , *AUTOANTIBODIES , *BIOMARKERS , *GENE expression , *INTERLEUKINS - Abstract
Abstract: Rheumatoid arthritis (RA) is a chronic and severe autoimmune disease that affects joint tissues, bone, and cartilage. However, the pathogenesis of RA is still unclear. Autoantibodies such as rheumatoid factor and anti-cyclic citrullinated peptide are useful tools for early diagnosis, monitoring disease activity, and predicting prognosis. Recently, many groups have focused their attention on the role of microRNAs in the pathogenesis of RA, as well as a potential biomarker to monitor RA. In fact, the expression of some microRNAs, such as miR-146a, is upregulated in different cell types and tissues in RA patients. MicroRNAs in RA could also be considered as possible future targets for new therapeutic approaches. [Copyright &y& Elsevier]
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- 2011
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30. Racial differences in systemic sclerosis disease presentation: A European Scleroderma Trials and Research group study
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Jaeger, Veronika K, Tikly, Mohammed, Dong, Xu, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Mengtao, Li, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich, A, Randone, Sb, Bannert, B, Iannone, F, Maurer, B, Jordan, S, Dobrota, R, Becker, M, Mihai, C, Becvarare, R, Tomčík, M, Bielecka, Ok, Gindzienska-Sieskiewicz, E, Karaszewska, K, Cutolo, M, Pizzorni, C, Paolino, S, Sulli, A, Ruaro, B, Alessandri, E, Riccardi, A, Giacco, V, Messitini, V, Irace, R, Kedor, C, Casteleyn, V, Hilger, J, Hoeppner, J, Rednic, S, Szabo, I, Petcu, A, Avouac, J, Camelia, F, Desbas, C, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Cavagna, L, Stork, J, Inanc, M, Joven, Be, Novak, S, Anic, F, Varju, C, Minier, T, Chizzolini, C, Allai, D, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Dolnicar, As, Coleiro, B, Gabrielli, A, Manfredi, L, Benfaremo, D, Ferrarini, A, Bancel, Df, Hij, A, Lansiaux, P, Lazzaroni, Mg, Hesselstrand, R, Wuttge, D, Andréasson, R, Martinovic, D, Bozic, I, Radic, M, Braun-Moscovici, Y, Monaco, Al, Furini, F, Hunzelmann, N, Moinzadeh, P, Pellerito, R, Caimmi, C, Bertoldo, E, Morovic-Vergles, J, Culo, Im, Pecher, Ac, Santamaria, Vo, Heitmann, S, Codagnone, M, Pflugfelder, J, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Hasler, P, Kretschmar, S, Kohm, M, Bajocchi, G, Salvador, Mj, Silva, Japd, Stamenkovic, B, Stankovic, A, Selmi, Cf, Santis, M, Ceribelli, A, Garzanova, L, Koneva, O, Starovoytova, M, Herrick, A, Puppo, F, Negrini, S, Murdaca, G, Engelhart, M, Szücs, G, Szamosi, S, de la Puente, C, Grande, Cs, Villanueva, Mjg, Midtvedt, Sø, Hoffmann-Vold, Am, Launay, D, Sobanski, V, Riccieri, V, Vasile, M, Ionescu, Rm, Opris, D, Sha, A, Woods, A, Gheorghiu, Am, Bojinca, M, Sunderkötter, C, Ehrchen, J, Ingegnoli, F, Mouthon, L, Dunogue, B, Chaigne, B, Legendre, P, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, von Mühlen CA, Pozzi, Mr, Eyerich, K, Lauffer, F, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Vanthuyne, M, Frédéric, H, Alegre-Sancho, Jj, Aringer, M, Herrmann, K, Günther, C, Westhovens, R, Langhe, E, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Üprus, M, Otsa, K, Yavuz, S, Granel, B, Radominski, Sc, De, C, Müller, S, Azevedo, Vf, Mendoza, F, Busquets, J, Popa, S, Agachi, S, Zenone, T, Pileckyte, M, Stebbings, S, Mathieu, A, Vacca, A, Sampaio-Barros, Pd, Stamp, L, Solanki, K, Silva, C, Schollum, J, Barns-Graham, H, Veale, D, O'Rourke, M, Loyo, E, Tineo, C, Paulino, G, Mohamed, Waaa, Rosato, E, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Visalli, E, Benenati, A, Amato, G, Ancuta, C, Villiger, P, Adler, S, Fröhlich, J, Kayser, C, Eduardo, Al, Fathi, N, Alii, S, Ahmed, M, Hasaneen, S, Hakeem, Ee, de la PG, Lefebvre, P, Martin, Jjg, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Galdo, Fd, Abignano, G, Eng, S, Seskute, G, Butrimiene, I, Rugiene, R, Karpec, D, Pascal, M, Kerzberg, E, Bianchi, W, Bianchi, Bv, Bianchi, Dv, Barcellos, Y, Castellví, I, Millan, M, Limonta, M, Rimar, D, Rosner, I, Slobodin, G, Couto, M, Spertini, F, Ribi, C, Buss, G, Marcoccia, A, Bondanini, F, Ciani, A, Kahl, S, Hsu, Vm, Martin, T, Poindron, V, Meghit, K, Moiseev, S, Novikov, P, Chung, L, Kolstad, K, Stark, M, Schmeiser, T, Thiele, A, Majewski, D, Zdrojewski, Z, Zaneta, S, Wierzba, K, Martínez-Barrio, J, López-Longo, Fj, Bernardino, V, Moraes-Fontes, Mf, Rodrigues, Ac, Riemekasten, G, Sommerlatte, S, Jendreck, S, Arnold, S, Levy, Y, Rezus, E, Cardoneanu, A, Burlui, Am, Pamuk, On, Puttini, Ps, Talotta, R, Bongiovanni, S, Poormoghim, H, Andalib, E, Almasi, S, Kötter, I, Krusche, M, Cuomo, G, Danzo, F, Masini, F, Gaches, F, Michaud, M, Cartos, F, Belloli, L, Casu, C, Sfikakis, P, Tektonidou, M, Furst, D, Feldman, Gr, Ramazan, Am, Nurmambet, E, Miroto, A, Suta, C, Andronache, I, Huizinga, Twj, de Vries-Bouwstra, J., Chizzolini, Carlo, Jaeger, Veronika K, Tikly, Mohammed, Xu, Dong, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Li, Mengtao, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich A, University of Zurich, Cerinic, Marco Matucci, Walker Ulrich, A, Randone, Silvia Bellando, Bannert, Bettina, Iannone, Florenzoaa, Maurer, Brittaab, Jordan, Suzanaab, Dobrota, Rucsandraab, Becker, Mikeab, Mihai, Carinaa, Becvarare, Radima, Tomcik, Michala, Bielecka, Otylia Kowala, Gindzienska-Sieskiewicz, Ewaa, Karaszewska, Katarzynaa, Cutolo, Maurizioa, Pizzorni, Carmena, Paolino, Sabrinaae, Sulli, Albertoa, Ruaro, Barbara, Alessandri, Elisa, Riccardi, Antonella, Giacco, Veronica, Messitini, Valentina, Irace, Rosaria, Kedor, Claudia, Casteleyn, Vincent, Hilger, Julia, Hoeppner, Jakob, Rednic, Simona, Szabo, Iulia, Petcu, Ana, Avouac, Jérome, Camelia, Frantz, Desbas, Carole, Vlachoyiannopoulos, Panayioti, Montecucco, Carlo Maurizio, Caporali, Roberto, Cavagna, Lorenzo, Stork, Jiri, Inanc, Murat, Joven, Beatriz E., Novak, Srdan, Anic, Felina, Varju, Cecilia, Minier, Tunde, Allai, Daniela, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Dolnicar, Alenka Sipek, Coleiro, Bernard, Gabrielli, Armando, Manfredi, Lucia, Benfaremo, Devi, Ferrarini, Alessia, Bancel, Dominique Farge, Hij, Adrian, Lazzaroni, Maria Grazia, Hesselstrand, Roger, Wuttge, Dirk, Andréasson, Kristofer, Martinovic, Duska, Bozic, Ivona, Radic, Mislav, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Furini, Federica, Hunzelmann, Nicola, Moinzadeh, Pia, Pellerito, Raffaele, Caimmi, Cristian, Bertoldo, Eugenia, Morovic-Vergles, Jadranka, Culo, Ivana Melanie, Pecher, Ann-Christian, Santamaria, Vera Ortiz, Heitmann, Stefan, Codagnone, Medeleine, Pflugfelder, Johanne, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthia, Hasler, Paul, Kretschmar, Samuel, Kohm, Michaela, Bajocchi, Gianluigi, Salvador, Maria João, Da Silva, JoséAntonio Pereira, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Ceribelli, Angela, Garzanova, Ludmila, Koneva, Olga, Starovoytova, Maya, Herrick, Ariane, Puppo, Francesco, Negrini, Simone, Murdaca, Giuseppe, Engelhart, Merete, Szücs, Gabriela, Szamosi, Szilvia, De La Puente, Carlo, Grande, Cristina Sobrino, Villanueva, Maria Jesus Garcia, Midtve, Øyvindbw, Hoffmann-Vold, Anna-Mariabw, Launay, Davidbx, Sobanski, Vincentbx, Riccieri, Valeriaby, Vasile, Massimilianoby, Stefantoni, Katia, Ionescu, Ruxandra Maria, Opris, Daniela, Sha, Ami, Woods, Adrianne, Gheorghiu, Ana Maria, Bojinca, Mihai, Sunderkötter, Cord, Ehrchen, Jan, Ingegnoli, Francesca, Mouthon, Luc, Dunogue, Bertrand, Chaigne, Benjamin, Legendre, Paul, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Von Mühlen, Carlos Alberto, Pozzi, Maria Rosa, Eyerich, Kilian, Lauffer, Felix, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Vanthuyne, Marie, Frédéric, Houssiau, Alegre-Sancho, Juan Jose, Aringer, Martin, Herrmann, Kristine, Günther, Claudia, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastião Cezar, De Souza Müller, Carolina, Feijóazevedo, Valderílio, Mendoza, Fabian, Busquets, Joanna, Popa, Sergei, Agachi, Svetlana, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Jordan, Sarah, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Silva, Cherumi, Schollum, Joanne, Barns-Graham, Helen, Veale, Dougla, O'Rourke, Marie, Loyo, Esthela, Tineo, Carmen, Paulino, Glenny, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Fröhlich, Johanne, Kayser, Cristiane, Eduardo, Andrade Lui, Fathi, Nihal, Alii, Safa, Ahmed, Marrow, Hasaneen, Samar, El Hakeem, Eman, De La Peña Lefebvre, Paloma García, Martin, Jorge Juan Gonzalez, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Del Galdo, Francesco, Abignano, Giuseppina, Eng, Sookho, Seskute, Goda, Butrimiene, Irena, Rugiene, Rita, Karpec, Diana, Pascal, Melanie, Kerzberg, Eduardo, Bianchi, Washington, Bianchi, Breno Valdetaro, Bianchi, Dante Valdetaro, Barcellos, Yeda, Castellví, Ivan, Millan, Milena, Limonta, Massimiliano, Rimar, Doron, Rosner, Itzhak, Slobodin, Gleb, Couto, Maura, Spertini, Françoi, Ribi, Camillo, Buss, Guillaume, Marcoccia, Antonella, Bondanini, Francesco, Ciani, Aldo, Kahl, Sarah, Hsu, Vivien M., Martin, Thierry, Poindron, Vincent, Meghit, Kilifa, Moiseev, Sergey, Novikov, Pavel, Chung, Lori, Kolstad, Kathleen, Stark, Marianna, Schmeiser, Tim, Thiele, Astrid, Majewski, Dominik, Zdrojewski, Zbigniew, Zaneta, Smolenska, Wierzba, Karol, Martínez-Barrio, Julia, López-Longo, Francisco Javier, Bernardino, Vera, Moraes-Fontes, Maria Francisca, Rodrigues, Ana Catarina, Riemekasten, Gabriela, Sommerlatte, Sabine, Jendreck, Sebastian, Arnold, Sabrina, Levy, Yair, Rezus, Elena, Cardoneanu, Anca, Burlui, Alexandra Maria, Pamuk, Omer Nuri, Puttini, Piercarlo Sarzi, Talotta, Rossella, Bongiovanni, Sara, Poormoghim, Hadi, Andalib, Elham, Almasi, Simin, Kötter, Ina, Krusche, Matrin, Cuomo, Giovanna, Danzo, Fiammetta, Masini, Francesco, Gaches, Franci, Michaud, Martin, Cartos, Florian, Belloli, Laura, Casu, Cinzia, Sfikakis, Petro, Tektonidou, Maria, Furst, Daniel, Feldman, Gary R., Ramazan, Ana-Maria, Nurmambet, Emel, Miroto, Amalia, Suta, Cristina, Andronache, Iulia, Huizinga, Tom W. J., De Vries-Bouwstra, Jeska, and Walker, Ulrich A.
- Subjects
Male ,Vital capacity ,Organ manifestations ,systemic sclerosis ,Type I ,race difference ,Systemic scleroderma ,Gastroenterology ,Scleroderma ,immunology ,0302 clinical medicine ,Diffusing capacity ,middle aged ,pulmonary hypertension ,Medicine ,Pharmacology (medical) ,030212 general & internal medicine ,organ manifestations ,races ,skin and connective tissue diseases ,Lung ,race ,pathophysiology ,African Continental Ancestry Group ,ddc:616 ,integumentary system ,disease course ,Hazard ratio ,Races ,10051 Rheumatology Clinic and Institute of Physical Medicine ,Pulmonary ,Middle Aged ,Blacks ,cohort analysis ,Autoantibodie ,3. Good health ,Asians ,female ,priority journal ,DNA Topoisomerases, Type I ,Black ,centromere ,Cohort ,Hypertension ,organ manifestation ,Systemic sclerosis ,Female ,systemic sclerosi ,Human ,Adult ,Asian Continental Ancestry Group ,medicine.medical_specialty ,Hypertension, Pulmonary ,European Continental Ancestry Group ,Black People ,610 Medicine & health ,complication ,Caucasian ,White People ,Article ,lung ,03 medical and health sciences ,Black person ,Rheumatology ,Asian People ,forced vital capacity ,Internal medicine ,geographic distribution ,Humans ,controlled study ,human ,DNA topoisomerase ,Aged ,Autoantibodies ,030203 arthritis & rheumatology ,Scleroderma, Systemic ,Asian ,business.industry ,Whites ,Systemic ,Odds ratio ,medicine.disease ,Pulmonary hypertension ,major clinical study ,mortality ,clinical feature ,business ,DNA Topoisomerases ,autoantibody - Abstract
Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.
- Published
- 2020
31. The clinical phenotype of Systemic Sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort
- Author
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Vanessa Smith, Ellen De Langhe, Elisabetta Zanatta, Jorge Rojas-Serrano, Christopher P. Denton, Roger Hesselstrand, Lorenzo Cavagna, Franco Franceschini, Montserrat-Ixchel Gonzalez-Perez, Angela Ceribelli, Konstantinos Triantafyllias, Marie-Elise Truchetet, Federica Furini, Giacomo De Luca, Alessio Furloni, Francesco Del Galdo, Yannick Allanore, Ilaria Cavazzana, Hadi Poormoghim, Corrado Campochiaro, G. Abignano, M. G. Lazzaroni, Francesca Ingegnoli, Paolo Airò, Silvia Bellando-Randone, Emiliano Marasco, Eugenia Bertoldo, Enrico Colombo, Eric Hachulla, S. Zingarelli, Oliver Distler, Jeska DeVries-Bouwstra, Simone Barsotti, Lazzaroni, Maria-Grazia, Marasco, Emiliano, Campochiaro, Corrado, DeVries-Bouwstra, Jeska, Gonzalez-Perez, Montserrat-Ixchel, Rojas-Serrano, Jorge, Hachulla, Eric, Zanatta, Elisabetta, Barsotti, Simone, Furini, Federica, Triantafyllias, Konstantino, Abignano, Giuseppina, Truchetet, Marie-Elise, De Luca, Giacomo, De Langhe, Ellen, Hesselstrand, Roger, Ingegnoli, Francesca, Bertoldo, Eugenia, Smith, Vanessa, Bellando-Randone, Silvia, Poormoghim, Hadi, Colombo, Enrico, Ceribelli, Angela, Furloni, Alessio, Zingarelli, Stefania, Cavazzana, Ilaria, Franceschini, Franco, Del Galdo, Francesco, Denton, Christopher P, Cavagna, Lorenzo, Distler, Oliver, Allanore, Yannick, and Airò, Paolo
- Subjects
Adult ,Male ,Myositis and muscle disease ,medicine.medical_specialty ,Scleroderma Renal Crisis ,Laboratory Diagnosis ,Systemic scleroderma ,behavioral disciplines and activities ,Gastroenterology ,Scleroderma ,Rheumatology ,Calcinosis ,Internal medicine ,Scleroderma and related disorders, Myositis and muscle disease, Autoantigens and Autoantibodies, Biomarkers, Laboratory Diagnosis ,medicine ,Humans ,Pharmacology (medical) ,autoantigens and autoantibodies ,biomarkers ,laboratory diagnosis ,myositis and muscle disease ,scleroderma and related disorders ,Autoantibodies ,Europe ,Exoribonucleases ,Exosome Multienzyme Ribonuclease Complex ,Female ,Middle Aged ,Phenotype ,Retrospective Studies ,Scleroderma, Systemic ,Registries ,skin and connective tissue diseases ,integumentary system ,business.industry ,Systemic ,Autoantigens and Autoantibodies ,Autoantibody ,Interstitial lung disease ,Cancer ,Dermatomyositis ,medicine.disease ,Scleroderma and related disorders ,Cohort ,business ,Biomarkers - Abstract
Objective To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), malignancies, and functional outcome of interstitial lung disease (ILD). Methods (1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared with 7202 anti-PM/Scl−, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case–control study: additional data were collected for 165 anti-PM/Scl+ SSc patients (85 from the EUSTAR registry) and compared with 257 anti-PM/Scl− SSc controls, matched for sex, cutaneous subset, disease duration and age at SSc onset. Results Patients with isolated anti-PM/Scl+, as compared with anti-Pm/Scl−, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). The presence of muscle involvement was associated with a more severe disease phenotype. Although very frequent, ILD had a better functional outcome in cases than in controls. In patients with both anti-PM/Scl and SSc-specific antibodies, a higher frequency of typical SSc features than in those with isolated anti-PM/Scl was observed. Conclusion The analysis of the largest series of anti-PM/Scl+ SSc patients so far reported helps to delineate a specific clinical subset with muscle involvement, cutaneous DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.
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