42 results on '"Boki KA"'
Search Results
2. Attainment of EULAR/ERA-EDTA targets of therapy with current immunosuppressive regimens and adjustments in treatment: a multicentre, real-life observational study.
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Pappa M, Kosmetatou M, Pieta A, Nikoloudaki M, Liapis NM, Tsalapaki C, Chalkia A, Argyriou E, Dimitroulas T, Cheila M, Demirtzoglou G, Papagoras C, Goules A, Katsiari C, Vassilopoulos D, Sidiropoulos P, Boki KA, Sfikakis PP, Liapis G, Gakiopoulou H, Voulgari PV, Boumpas DT, Bertsias G, Tektonidou MG, and Fanouriakis A
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- Humans, Female, Male, Adult, Middle Aged, Treatment Outcome, Cyclophosphamide therapeutic use, Mycophenolic Acid therapeutic use, Young Adult, Immunosuppressive Agents therapeutic use, Lupus Nephritis drug therapy, Glomerular Filtration Rate
- Abstract
Objective: To estimate real-life European Alliance of Associations for Rheumatology (EULAR)/European Renal Association (ERA)-European Dialysis and Transplantation Association (EDTA) response rates and predictors for no response in patients with lupus nephritis (LN) managed with conventional immunosuppressive therapies., Methods: Ambidirectional cohort study of patients with new-onset LN (period 2014-to date). Response rates in the first year were calculated, and all treatment modifications were recorded. Univariate and multivariate regression analyses were performed to assess determinants of failure to respond at 12 months., Results: 140 patients were included (81.4% women, median (IQR) age at LN diagnosis 38 (22) years). Among them, 32.1% presented with nephrotic range proteinuria, 28.6% with glomerular filtration rate <60 mL/min, 76.6% had proliferative and 19.7% class V LN. Initial treatment consisted of cyclophosphamide in 51.4% of patients (84.7% high-dose, 15.3% low-dose) and mycophenolate in 32.1%. 120 patients had available data at 12 months. EULAR/ERA-EDTA renal response rates at 3, 6 and 12 months were achieved by 72.6%, 78.5% % and 69.2% of patients, respectively. In multivariate analysis, increased Chronicity Index at baseline was associated with failure to achieve either complete or partial response at 12 months (OR 2.26, 95% CI 1.35 to 3.77). Notably, 20% of patients required treatment modifications due to suboptimal response during the first 12 months, with the addition of or switch to a different immunosuppressive drug in seven and nine patients, respectively., Conclusions: More than two-thirds of patients with LN attain EULAR/ERA-EDTA response rates by 12 months, but 20% require therapy modifications within this time period. Patients with increased chronicity in baseline biopsy, when combined with histological activity, are at higher risk for a lack of clinical response., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2024
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3. Senescent cells in giant cell arteritis display an inflammatory phenotype participating in tissue injury via IL-6-dependent pathways.
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Veroutis D, Argyropoulou OD, Goules AV, Kambas K, Palamidas DA, Evangelou K, Havaki S, Polyzou A, Valakos D, Xingi E, Karatza E, Boki KA, Cavazza A, Kittas C, Thanos D, Ricordi C, Marvisi C, Muratore F, Galli E, Croci S, Salvarani C, Gorgoulis VG, and Tzioufas AG
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- Humans, Interleukin-6 genetics, Matrix Metalloproteinase 9 genetics, Endothelial Cells metabolism, Retrospective Studies, Phenotype, Cellular Senescence, Inflammation complications, Giant Cell Arteritis complications, Polymyalgia Rheumatica complications
- Abstract
Objectives: Age is the strongest risk factor of giant cell arteritis (GCA), implying a possible pathogenetic role of cellular senescence. To address this question, we applied an established senescence specific multimarker algorithm in temporal artery biopsies (TABs) of GCA patients., Methods: 75(+) TABs from GCA patients, 22(-) TABs from polymyalgia rheumatica (PMR) patients and 10(-) TABs from non-GCA/non-PMR patients were retrospectively retrieved and analysed. Synovial tissue specimens from patients with inflammatory arthritis and aorta tissue were used as disease control samples. Senescent cells and their histological origin were identified with specific cellular markers; IL-6 and MMP-9 were investigated as components of the senescent associated secretory phenotype by triple costaining. GCA or PMR artery culture supernatants were applied to fibroblasts, HUVECs and monocytes with or without IL-6R blocking agent to explore the induction of IL-6-associated cellular senescence., Results: Senescent cells were present in GCA arteries at higher proportion compared with PMR (9.50% vs 2.66%, respectively, p<0.0001) and were mainly originated from fibroblasts, macrophages and endothelial cells. IL-6 was expressed by senescent fibroblasts, and macrophages while MMP-9 by senescent fibroblasts only. IL-6(+) senescent cells were associated with the extension of vascular inflammation (transmural inflammation vs adventitia limited disease: 10.02% vs 4.37%, respectively, p<0.0001). GCA but not PMR artery culture supernatant could induce IL-6-associated senescence that was partially inhibited by IL-6R blockade., Conclusions: Senescent cells with inflammatory phenotype are present in GCA arteries and are associated with the tissue inflammatory bulk, suggesting a potential implication in disease pathogenesis., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2024
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4. Patterns and factors associated with pneumococcal vaccination in a prospective cohort of 1,697 patients with rheumatoid arthritis.
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Thomas K, Lazarini A, Kaltsonoudis E, Voulgari PV, Drosos AA, Repa A, Sali AMI, Sidiropoulos P, Tsatsani P, Gazi S, Fragkiadaki K, Tektonidou MG, Sfikakis PP, Katsimbri P, Boumpas D, Argyriou E, Boki KA, Karagianni K, Katsiari C, Evangelatos G, Iliopoulos A, Grika EP, Vlachoyiannopoulos PG, Dimitroulas T, Garyfallos A, Melissaropoulos K, Georgiou P, Georganas C, Vounotrypidis P, Ntelis K, Areti M, Kitas GD, and Vassilopoulos D
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Introduction: Patients with rheumatoid arthritis (RA) are at increased risk for serious infections. Pneumococcal vaccination is among the most important preventive measures, however, vaccine uptake is suboptimal. We explored the rate and factors associated with pneumococcal vaccination in a contemporary RA cohort., Materials and Methods: Multi-center, prospective, RA cohort study in Greece. Patient and disease characteristics and influenza and pneumococcal vaccinations were documented at baseline and 3 years later., Results: One thousand six hundred and ninety-seven patients were included and 34.5% had already received at least one pneumococcal vaccine at baseline. Among 1,111 non-vaccinated patients, 40.1% received pneumococcal vaccination during follow-up, increasing the vaccine coverage to 60.8%. By multivariate analysis, positive predictors for pneumococcal vaccination included prescription of influenza vaccine (OR = 33.35, 95% CI: 18.58-59.85), history of cancer (OR = 2.35, 95% CI: 1.09-5.06), bDMARD use (OR = 1.85, 95% CI: 1.29-2.65), seropositivity (OR = 1.47, 95% CI: 1.05-2.05), and high disease activity (DAS28-ESR, OR = 1.33, 95% CI: 1.17-1.51). Male sex (OR = 0.65, 95% CI: 0.43-0.99) was a negative predictor for pneumococcal vaccination during follow-up., Discussion: Despite increasing rates of pneumococcal vaccine coverage, 40% of RA patients remain unvaccinated. Severe disease, bDMARD use, comorbidities, and more importantly flu vaccination were the most significant factors associated with pneumococcal vaccination, emphasizing the currently unmet need for cultivating a "vaccination culture" in RA patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Thomas, Lazarini, Kaltsonoudis, Voulgari, Drosos, Repa, Sali, Sidiropoulos, Tsatsani, Gazi, Fragkiadaki, Tektonidou, Sfikakis, Katsimbri, Boumpas, Argyriou, Boki, Karagianni, Katsiari, Evangelatos, Iliopoulos, Grika, Vlachoyiannopoulos, Dimitroulas, Garyfallos, Melissaropoulos, Georgiou, Georganas, Vounotrypidis, Ntelis, Areti, Kitas and Vassilopoulos.)
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- 2023
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5. Idiopathic retroperitoneal fibrosis: clinical features, treatment modalities, relapse rate in Greek patients and a review of the literature.
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Zampeli E, Venetsanopoulou AI, Christaki S, Argyropoulou OD, Boki KA, Manoussakis MN, Skopouli FN, Tzioufas AG, and Moutsopoulos HM
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- Acute-Phase Proteins, Chronic Disease, Female, Greece, Humans, Immunoglobulin G, Male, Recurrence, Retrospective Studies, Retroperitoneal Fibrosis diagnostic imaging, Retroperitoneal Fibrosis drug therapy
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Objectives: Retroperitoneal fibrosis (RPF) is mostly idiopathic (iRPF); however, it can be secondary to drugs, malignancies, infections, or, as recently recognised, can be part of the IgG4-related diseases. The aim of our study was i) to describe the presenting clinical/laboratory/imaging features and treatment modalities used in patients with iRPF and ii) to evaluate factors potentially associated with disease relapse., Methods: The medical records of patients diagnosed with iRPF and followed in four tertiary medical units in Athens, Greece from 2000 to 2018 were retrospectively evaluated., Results: Sixty-seven patients with iRPF were included in the study. Seventy-three per cent were males, with a mean age at diagnosis 56.0±9.2 years. Low-back pain (63%) and constitutional symptoms (57%) were the commonest presenting symptoms. Elevated acute-phase reactants (78%), anaemia (43%) and impaired renal function (41%) were the most common laboratory findings. Serum IgG4 at diagnosis was evaluated in 36/67 patients and 36% of them had elevated levels (mean 297.7±166.3mg/dL). Diagnosis was mainly based on abdominal CT and/or MRI. Clinical/laboratory/radiological presentation did not differ between patients with elevated and normal serum IgG4 levels. Steroids were used as first-line treatment in 98%. Relapse occurred in 28.6% after a mean of 43.1±31.8 months. Relapse did not associate to initial clinical/imaging findings or to any treatment used, however patients with increased serum IgG4 had a significantly higher relapse rate (75% vs. 25%, p=0.005)., Conclusions: Relapse occurred in one-fifth of patients independently of the initial clinical/radiographic presentation or treatment used. iRPF patients with baseline elevated serum IgG4 levels have a higher relapse rate.
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- 2022
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6. COVID-19 infection among autoimmune rheumatic disease patients: Data from an observational study and literature review.
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Bakasis AD, Mavragani CP, Boki KA, Tzioufas AG, Vlachoyiannopoulos PG, Stergiou IE, Skopouli FN, and Moutsopoulos HM
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- Adult, Aged, Aged, 80 and over, Antibodies, Viral biosynthesis, Asymptomatic Infections epidemiology, Autoimmune Diseases drug therapy, Autoimmune Diseases immunology, Comorbidity, Connective Tissue Diseases drug therapy, Connective Tissue Diseases immunology, Critical Illness, Female, Greece epidemiology, Hospitalization statistics & numerical data, Humans, Hypothyroidism epidemiology, Immunocompromised Host, Immunoglobulin G biosynthesis, Immunologic Factors adverse effects, Immunologic Factors therapeutic use, Immunosuppressive Agents adverse effects, Immunosuppressive Agents therapeutic use, Inflammation, Lung Diseases epidemiology, Male, Middle Aged, Observational Studies as Topic, Review Literature as Topic, Rheumatic Diseases drug therapy, Rheumatic Diseases epidemiology, Severity of Illness Index, Symptom Assessment, Autoimmune Diseases epidemiology, COVID-19 epidemiology, Connective Tissue Diseases epidemiology, SARS-CoV-2 immunology
- Abstract
The impact of SARS-CoV-2 infection in patients with autoimmune/auto-inflammatory rheumatic diseases (AARD) under immunomodulatory treatment has been a focus of interest during the COVID-19 pandemic. In this observational study, demographic data, disease related features and comorbidities, COVID-19 manifestations and outcome as well as antibody responses to SARS-CoV-2 were recorded among 77 consecutive patients with underlying AARD infected by SARS-CoV-2. Analysis of data was performed using univariate and multivariate models. Most patients (68.8%) had a mild COVID-19 course. The predominant clinical manifestations were fatigue (58.4%), low grade fever (45.4%) and upper respiratory tract symptoms (68.8%). About a quarter of patients required hospitalization (23.3%) and the mortality rate was 1.3%. Regarding COVID-19 severity, prior treatment with corticosteroids, mycophenolate mofetil or rituximab was more common in patients who developed a more serious disease course (60.0 vs 29.9%, p = 0.003, 40.0 vs 7.5%, p = 0.003, 10.0 vs 0.0%, p = 0.009, respectively). When disease related features and comorbidities were considered in multivariate models, older age and lung disease in the context of the AARD were found to be independent predictive factors for hospitalization (OR [95%]: 1.09 [1.03-1.15] and 6.43 [1.11-37.19]). Among COVID-19 related features, patients with shortness of breath and high-grade fever were more likely to get hospitalized (OR [95%]: 7.06 [1.36-36.57], 12.04 [2.96-48.86]), while anosmia was independently associated with lower hospitalization risk (OR [95%]: 0.09 [0.01-0.99]). Though the majority of AARD patients displayed a mild COVID-19 course, certain underlying disease features and COVID-19 related manifestations should prompt alertness for the physician to identify patients with AARD at high risk for severe COVID-19 and need for hospitalization., (Copyright © 2021 Elsevier Ltd. All rights reserved.)
- Published
- 2021
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7. Incidence, risk factors and validation of the RABBIT score for serious infections in a cohort of 1557 patients with rheumatoid arthritis.
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Thomas K, Lazarini A, Kaltsonoudis E, Voulgari PV, Drosos AA, Repa A, Sali AMI, Sidiropoulos P, Tsatsani P, Gazi S, Evangelia A, Boki KA, Katsimbri P, Boumpas D, Fragkiadaki K, Tektonidou MG, Sfikakis PP, Karagianni K, Sakkas LI, Grika EP, Vlachoyiannopoulos PG, Evangelatos G, Iliopoulos A, Dimitroulas T, Garyfallos A, Melissaropoulos K, Georgiou P, Areti M, Georganas C, Vounotrypidis P, Georgiopoulos G, Kitas GD, and Vassilopoulos D
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- Aged, Antirheumatic Agents therapeutic use, Arthritis, Rheumatoid drug therapy, Comorbidity, Female, Glucocorticoids therapeutic use, Humans, Incidence, Male, Middle Aged, Risk Factors, Arthritis, Rheumatoid epidemiology, Infections epidemiology, Opportunistic Infections epidemiology
- Abstract
Objectives: Predicting serious infections (SI) in patients with rheumatoid arthritis (RA) is crucial for the implementation of appropriate preventive measures. Here we aimed to identify risk factors for SI and to validate the RA Observation of Biologic Therapy (RABBIT) risk score in real-life settings., Methods: A multi-centre, prospective, RA cohort study in Greece. Demographics, disease characteristics, treatments and comorbidities were documented at first evaluation and one year later. The incidence of SI was recorded and compared with the expected SI rate using the RABBIT risk score., Results: A total of 1557 RA patients were included. During follow-up, 38 SI were recorded [incidence rate ratio (IRR): 2.3/100 patient-years]. Patients who developed SI had longer disease duration, higher HAQ at first evaluation and were more likely to have a history of previous SI, chronic lung disease, cardiovascular disease and chronic kidney disease. By multivariate analysis, longer disease duration (IRR: 1.05; 95% CI: 1.005, 1.1), history of previous SI (IRR: 4.15; 95% CI: 1.7, 10.1), diabetes (IRR: 2.55; 95% CI: 1.06, 6.14), chronic lung disease (IRR: 3.14; 95% CI: 1.35, 7.27) and daily prednisolone dose ≥10 mg (IRR: 4.77; 95% CI: 1.47, 15.5) were independent risk factors for SI. Using the RABBIT risk score in 1359 patients, the expected SI incidence rate was 1.71/100 patient-years, not different from the observed (1.91/100 patient-years; P = 0.97)., Conclusion: In this large real-life, prospective study of RA patients, the incidence of SI was 2.3/100 patient-years. Longer disease duration, history of previous SI, comorbidities and high glucocorticoid dose were independently associated with SI. The RABBIT score accurately predicted SI in our cohort., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
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- 2021
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8. Treatment patterns and achievement of the treat-to-target goals in a real-life rheumatoid arthritis patient cohort: data from 1317 patients.
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Thomas K, Lazarini A, Kaltsonoudis E, Drosos A, Papalopoulos I, Sidiropoulos P, Tsatsani P, Gazi S, Pantazi L, Boki KA, Katsimbri P, Boumpas D, Fragkiadaki K, Tektonidou M, Sfikakis PP, Karagianni K, Sakkas LI, Grika EP, Vlachoyiannopoulos PG, Evangelatos G, Iliopoulos A, Dimitroulas T, Garyfallos A, Melissaropoulos K, Georgiou P, Areti M, Georganas C, Vounotrypidis P, Kitas GD, and Vassilopoulos D
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Background: Data regarding the real-life predictors of low disease activity (LDA) in rheumatoid arthritis (RA) patients are limited. Our aim was to evaluate the rate and predictors of LDA and treatment patterns in RA., Methods: This was a multicenter, prospective, RA cohort study where patients were evaluated in two different time points approximately 12 months apart. Statistical analysis was performed in order to identify predictors of LDA while patterns of disease-modifying anti-rheumatic drug [DMARDs; conventional synthetic (csDMARD) or biologic (bDMARD)] and glucocorticoid (GC) use were also recorded., Results: The total number of patients included was 1317 (79% females, mean age: 62.9 years, mean disease duration: 10.3 years). After 1 year, 57% had achieved LDA (DAS28ESR<3.2) while 43% did not (34%: moderate disease activity: DAS28ESR ⩾3.2 to <5.1, 9%: high disease activity, DAS28ESR ⩾5.1). By multivariate analysis, male sex was positively associated with LDA [odds ratio (OR) = 2.29 p < 0.001] whereas advanced age (OR = 0.98, p = 0.005), high Health Assessment Questionnaire (HAQ) score (OR = 0.57, p < 0.001), use of GCs (OR = 0.75, p = 0.037) or ⩾2 bDMARDs (OR = 0.61, p = 0.002), high co-morbidity index (OR = 0.86, p = 0.011) and obesity (OR = 0.62, p = 0.002) were negative predictors of LDA. During follow-up, among active patients (DAS28ESR >3.2), 21% initiated (among csDMARDs users) and 22% switched (among bDMARDs users) their bDMARDs., Conclusion: In a real-life RA cohort, during 1 year of follow-up, 43% of patients do not reach treatment targets while only ~20% of those with active RA started or switched their bDMARDs. Male sex, younger age, lower HAQ, body mass index and co-morbidity index were independent factors associated with LDA while use of GCs or ⩾2 bDMARDs were negative predictors., Competing Interests: Conflict of interest statement: The authors declare that there is no conflict of interest., (© The Author(s), 2020.)
- Published
- 2020
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9. Low disease activity-irrespective of serologic status at baseline-associated with reduction of corticosteroid dose and number of flares in patients with systemic lupus erythematosus treated with belimumab: A real-life observational study.
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Fanouriakis A, Adamichou C, Koutsoviti S, Panopoulos S, Staveri C, Klagou A, Tsalapaki C, Pantazi L, Konsta S, Mavragani CP, Dimopoulou D, Ntali S, Katsikas G, Boki KA, Vassilopoulos D, Konstantopoulou P, Liossis SN, Elezoglou A, Tektonidou M, Sidiropoulos P, Erden A, Sfikakis PP, Bertsias G, and Boumpas DT
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- Adult, Drug Therapy, Combination, Female, Humans, Lupus Erythematosus, Systemic blood, Lupus Erythematosus, Systemic diagnosis, Male, Middle Aged, Prednisone therapeutic use, Prospective Studies, Severity of Illness Index, Treatment Outcome, Adrenal Cortex Hormones therapeutic use, Antibodies, Monoclonal, Humanized therapeutic use, Immunosuppressive Agents therapeutic use, Lupus Erythematosus, Systemic drug therapy
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Background: Low disease activity is a validated target of current systemic lupus erythematosus (SLE) therapy. The aim of this study was to assess the ability of belimumab to achieve low disease activity states in real-life settings., Methods: Multicentre prospective observational study of consecutive SLE patients receiving belimumab for at least 3 months, due to active disease refractory to at least one conventional immunosuppressant. Disease activity, including the recently defined lupus low disease activity state (LLDAS) and remission (clinical SLEDAI-2K = 0), accrual of organ damage, flares and side effects were documented., Results: Ninety-one patients were included [94.5% women, mean (SD) age 45.9 (12.5) years]. Most frequent manifestations were arthritis (76.7%), rash (72.5%), serologic activity (low C3/C4 and/or high anti-dsDNA; 54.9%), hair loss (47.2%) and mucosal ulcers (27.5%). Median (range) duration of treatment was 10.5 (3.0-42.1) months. Belimumab significantly decreased average SLEDAI-2K, physician global assessment (PGA) and daily prednisone dose over time, as early as 3 months after initiation, with over 20% of patients discontinuing corticosteroids. Although reduction in clinical (i.e., excluding serology) SLEDAI-2K was more pronounced in patients who were serologically active (from 8 to 1.5 at 12 months) as compared to serologically inactive (from 6 to 4) at baseline, attainment of LLDAS did not differ between the two groups and was reached by more than 40% of completer patients after 9-12 months. In addition, the number of flares and severe flares was reduced by 62% and 50%, respectively, during the first 12 months of treatment. Twenty patients (22.0%) discontinued treatment due to inadequate response and two due to side effects potentially related to the drug., Conclusions: In real-life, belimumab is efficacious in achieving low disease activity in over 40% of unselected patients, in combination with reduction of corticosteroid dosage and number of flares. Both serologically active and inactive patients respond to the drug., (Copyright © 2018 Elsevier Inc. All rights reserved.)
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- 2018
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10. Five-year prospective multi-center cohort study of patients with giant cell arteritis in Greece.
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Tsalapaki C, Nikitopoulou E, Boki KA, Boumpas D, Sfikakis PP, Vosvotekas G, Voulgari PV, and Vassilopoulos D
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Giant cell arteritis (GCA) is the most common systemic vasculitis in the aged population associated with significant morbidity due to the long term administration of corticosteroids and the presence of various comorbidities. Data regarding its current treatment modalities, comorbidities, morbidity and mortality in Greece are limited. In this multi-center, prospective study that begun at the end of 2015 patients with newly diagnosed GCA according to the modified 1990 ACR criteria, as well as individuals with established or relapsing disease have been included. During the 1
st phase of the study that is still ongoing, data are being collected concerning demographic and clinical characteristics of the patients, treatment at the onset of the disease and at relapses, relapses, adverse events of therapy, comorbidities, hospitalizations and deaths. During the 2nd and 3rd phase of the study patients will be reevaluated 2 and 5 years after their 1st evaluation. The study is expected to provide valuable data regarding the current clinical characteristics, comorbidities, therapeutic regimens used, relapse rate, morbidity and mortality of patients with GCA., (© 2018 The Mediterranean Journal of Rheumatology (MJR).)- Published
- 2018
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11. Comparative Analysis and Predictors of 10-year Tumor Necrosis Factor Inhibitors Drug Survival in Patients with Spondyloarthritis: First-year Response Predicts Longterm Drug Persistence.
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Flouri ID, Markatseli TE, Boki KA, Papadopoulos I, Skopouli FN, Voulgari PV, Settas L, Zisopoulos D, Iliopoulos A, Geborek P, Drosos AA, Boumpas DT, and Sidiropoulos P
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- Adalimumab therapeutic use, Adult, Female, Humans, Infliximab therapeutic use, Male, Middle Aged, Prospective Studies, Registries, Treatment Outcome, Antirheumatic Agents therapeutic use, Arthritis, Psoriatic drug therapy, Biological Products therapeutic use, Spondylarthritis drug therapy, Tumor Necrosis Factor-alpha antagonists & inhibitors
- Abstract
Objective: To evaluate the 10-year drug survival of the first tumor necrosis factor inhibitor (TNFi) administered to patients with spondyloarthritis (SpA) overall and comparatively between SpA subsets, and to identify predictors of drug retention., Methods: Patients with SpA in the Hellenic Registry of Biologic Therapies, a prospective multicenter observational cohort, starting their first TNFi between 2004-2014 were analyzed. Kaplan-Meier curves and Cox regression models were used., Results: Overall, 404 out of 1077 patients (37.5%) discontinued treatment (followup: 4288 patient-yrs). Ten-year drug survival was 49%. In the unadjusted analyses, higher TNFi survival was observed in patients with ankylosing spondylitis (AS) compared to undifferentiated SpA and psoriatic arthritis [PsA; significant beyond the first 2.5 (p = 0.003) years and 7 years (p < 0.001), respectively], and in patients treated for isolated axial versus peripheral arthritis (p = 0.001). In all multivariable analyses, male sex was a predictor for longer TNFi survival. Use of methotrexate (MTX) was a predictor in PsA and in patients with peripheral arthritis. Absence of peripheral arthritis and use of a monoclonal antibody (as opposed to non-antibody TNFi) independently predicted longer TNFi survival in axial disease because of lower rates of inefficacy. Achievement of major responses during the first year in either axial or peripheral arthritis was the strongest predictor of longer therapy retention (HR 0.33, 95% CI 0.26-0.41 for Ankylosing Spondylitis Disease Activity Score inactive disease, and HR 0.35, 95% CI 0.24-0.50 for 28-joint Disease Activity Score remission)., Conclusion: The longterm retention of the first TNFi administered to patients with SpA is high, especially for males with axial disease. The strongest predictor of longterm TNFi survival is a major response within the first year of treatment.
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- 2018
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12. Multicenter Cross-sectional Study of Patients with Rheumatoid Arthritis in Greece: Results from a cohort of 2.491 patients.
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Thomas K, Lazarini A, Kaltsonoudis E, Drosos A, Papalopoulos I, Sidiropoulos P, Katsimbri P, Boumpas D, Tsatsani P, Gazi S, Fragkiadaki K, Tektonidou M, Sfikakis PP, Pantazi L, Boki KA, Grika EP, Vlachoyiannopoulos PG, Karagianni K, Sakkas LI, Dimitroulas T, Garyfallos A, Kassimos D, Evangelatos G, Iliopoulos A, Areti M, Georganas C, Melissaropoulos K, Georgiou P, Vounotrypidis P, Ntelis K, Mavragani CP, Bournazos I, Katsifis G, Mavrommatis C, Kitas GD, and Vassilopoulos D
- Abstract
Aim of the Study: To evaluate the current disease characteristics, treatment and comorbidities of rheumatoid arthritis (RA) in Greece., Methods: Multicenter, cross-sectional study with a 9-month recruitment period between 2015 and 2016. Demographics, disease characteristics, treatment and comorbidities were collected via a web-based platform., Results: 2.491 RA patients were recruited: 96% from tertiary referral centers, 79% were females with a mean age of 63.1 years and disease duration of 9.9 years. Fifty-two percent were rheumatoid factor and/or anti-CCP positive, while 41% had erosive disease. Regarding treatment, 82% were on conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs), 42% on biologic DMARDs (TNFi: 22%, non-TNFi: 20%) and 40% on corticosteroids (mean daily dose: 5.2 mg). Despite therapy, 36% of patients had moderate and 12% high disease activity. The most frequent comorbidities were hypertension (42%), hyperlipidemia (33%), osteoporosis (29%), diabetes mellitus (15%) and depression (12%). Latent tuberculosis infection (positive tuberculin skin test or interferon gamma release assay) was diagnosed in 13 and 15.3% of patients, respectively. Regarding chronic viral infections, 6.2% had history of herpes zoster while 2% and 0.7% had chronic hepatitis B and C virus infection, respectively. A history of serious infection was documented in 9.6%. Only 36% and 52% of the participants had ever been vaccinated against pneumococcus and influenza virus, respectively., Conclusion: This is one of the largest epidemiologic studies providing valuable data regarding the current RA characteristics in Greece. Half of patients were seropositive but despite therapy, half displayed residual disease activity, while preventive vaccination was limited., (© 2018 The Mediterranean Journal of Rheumatology (MJR).)
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- 2018
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13. Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
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Mavrogeni S, Markousis-Mavrogenis G, Koutsogeorgopoulou L, Dimitroulas T, Bratis K, Kitas GD, Sfikakis P, Tektonidou M, Karabela G, Stavropoulos E, Katsifis G, Boki KA, Kitsiou A, Filaditaki V, Gialafos E, Plastiras S, Vartela V, and Kolovou G
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- Adult, Echocardiography methods, Electrocardiography methods, Female, Follow-Up Studies, Humans, Male, Middle Aged, Treatment Outcome, Connective Tissue Diseases diagnostic imaging, Connective Tissue Diseases physiopathology, Magnetic Resonance Imaging, Cine methods
- Abstract
Background-Aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis., Patients-Methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Acute and chronic lesions were assessed by T2>2 with positive LGE and T2<2 with positive LGE, respectively., Results: In 3/5 TA, 3/4 CSS, 4/5 WG, 10/16 SLE, 9/12 RA, 6/8 MCTD, 4/12 AS, 1/3 PMR, 2/8 SSc and 2/5 DM, the T2 ratio was higher compared to normal (2.78±0.25 vs 1.5±0.2, p<0.01). Myocarditis was identified in 1 TA, 1 SLE, 1 RA, 1 SSc and 2 DM patients; diffuse, subendocardial fibrosis in 1 CSS and 1 RA patient, while subendocardial myocardial infarction in 3 SLE, 1 MCTD, 1 PMR and 2 RA patients. CMR re-evaluation after 6 and 12months of rheumatic and cardiac treatment, available in 28/52 CTDs with increased T2 ratio, showed significant improvement in T2 ratio (p<0.001), non-significant change in LGE extent and normalisation of those with impaired LV function., Conclusions: Occult CMR lesions, including oedema, myocarditis, diffuse subendocardial fibrosis and myocardial infarction are not unusual in treatment naïve CTDs and may be reversed with appropriate treatment., (Copyright © 2017 Elsevier B.V. All rights reserved.)
- Published
- 2017
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14. Innate immunity alterations in idiopathic interstitial pneumonias and rheumatoid arthritis-associated interstitial lung diseases.
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Papanikolaou IC, Boki KA, Giamarellos-Bourboulis EJ, Kotsaki A, Kagouridis K, Karagiannidis N, and Polychronopoulos VS
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- Adult, Aged, Arthritis, Rheumatoid metabolism, Bronchoalveolar Lavage Fluid chemistry, Bronchoalveolar Lavage Fluid cytology, Bronchoalveolar Lavage Fluid immunology, Cells, Cultured, Female, Flow Cytometry, Humans, Idiopathic Interstitial Pneumonias metabolism, Killer Cells, Natural immunology, Killer Cells, Natural metabolism, Leukocytes, Mononuclear immunology, Leukocytes, Mononuclear metabolism, Lung immunology, Lung metabolism, Lung pathology, Lung Diseases, Interstitial metabolism, Male, Membrane Glycoproteins immunology, Membrane Glycoproteins metabolism, Middle Aged, Natural Killer T-Cells immunology, Natural Killer T-Cells metabolism, Prospective Studies, Pulmonary Fibrosis immunology, Pulmonary Fibrosis metabolism, Receptors, Immunologic immunology, Receptors, Immunologic metabolism, Toll-Like Receptor 2 immunology, Toll-Like Receptor 2 metabolism, Toll-Like Receptor 4 immunology, Toll-Like Receptor 4 metabolism, Triggering Receptor Expressed on Myeloid Cells-1, Tumor Necrosis Factor-alpha immunology, Tumor Necrosis Factor-alpha metabolism, Arthritis, Rheumatoid immunology, Idiopathic Interstitial Pneumonias immunology, Immunity, Innate immunology, Lung Diseases, Interstitial immunology
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Background: This is a prospective cohort study elucidating innate immunity in idiopathic pulmonary fibrosis (IPF), cryptogenic organizing pneumonia (COP), rheumatoid arthritis-associated usual interstitial pneumonia (RA-UIP) and RA-associated non specific interstitial pneumonia (RA-NSIP)., Methods: 23 IPF subjects, 9 COP subjects, 5 RA-UIP subjects, 8 RA-NSIP subjects were enrolled. 10 subjects were excluded. 19 healthy subjects served as controls. Blood and bronchoalveolar lavage (BAL) were obtained. Natural killer (NK) and NKT cells, NK cells apoptosis and the expression of triggering receptor expressed on myeloid cells type 1 (TREM-1) were assessed. Tumor necrosis factor-α (TNF-α) production was measured in cell cultures after stimulation with lipopolysaccharide endotoxin (LPS) and Pam3CysSK3, and in BAL. Surface expression of Toll-like receptors (TLR) 2 and 4 on peripheral blood monocytes (PBMC's) and circulating NK cells was also assessed., Results: RA-NSIP had low blood NKs, marginally insignificant (p=0.07). These NKs poorly produced TNF-α after LPS stimulation. TLR's expression on NK cells was similar throughout disease groups and controls. PBMC's mainly from IPF patients exhibited low TNF-α production after LPS stimulation but not after Pam3CysSK3 stimulation, while TLR4 expression on PBMC's was found normal in all study groups. TLR2 expression on PBMC's was increased in IPF, but mainly in COP, RA-UIP and RA-NSIP (p=0.015). TREM-1 expression was significant on COP monocytes and on COP neutrophils versus controls. RA-NSIP monocytes also exhibited TREM-1 expression (p=0.07). Decreased TNF-α concentration in BAL was finally observed in IPF and RA-UIP., Conclusions: Innate immunity in the lungs and the peripheral circulation in IPF and RA-UIP are similar and more fibrotic than in RA-NSIP which is characterized by NK cell depletion and dysfunction. TREM-1 and TLR's likely affect patterns of inflammation in various interstitial lung diseases., (Copyright © 2014 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.)
- Published
- 2015
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15. Comparative effectiveness and survival of infliximab, adalimumab, and etanercept for rheumatoid arthritis patients in the Hellenic Registry of Biologics: Low rates of remission and 5-year drug survival.
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Flouri I, Markatseli TE, Voulgari PV, Boki KA, Papadopoulos I, Settas L, Zisopoulos D, Skopouli FN, Iliopoulos A, Bertsias GK, Geborek P, Drosos AA, Boumpas DT, and Sidiropoulos P
- Subjects
- Adalimumab, Adult, Aged, Etanercept, Female, Follow-Up Studies, Humans, Infliximab, Male, Middle Aged, Prospective Studies, Registries, Severity of Illness Index, Treatment Outcome, Antibodies, Monoclonal therapeutic use, Antibodies, Monoclonal, Humanized therapeutic use, Antirheumatic Agents therapeutic use, Arthritis, Rheumatoid drug therapy, Immunoglobulin G therapeutic use, Receptors, Tumor Necrosis Factor therapeutic use
- Abstract
Objective: To compare effectiveness, drug survival, and safety between infliximab, adalimumab, and etanercept, in a nationwide cohort of rheumatoid arthritis (RA) patients., Methods: This study is a prospective cohort study of 1208 active RA patients. Effectiveness, drug survival, and serious adverse events during entire follow-up (median 2.9 years) were monitored., Results: EULAR and CDAI responses were comparable between the three agents (EULAR good/moderate responses at 12 months ranged 76-79%). At 12 months, 15-23% achieved remission. For adalimumab and etanercept, adjusted hazard rate (HR) for EULAR/ACR remission (reference: infliximab) was 2.7 and 2.1 (95% confidence interval was 1.7-4.1 and 1.3-3.4, respectively); males (HR 1.6; 1.1-2.4), use of glucocorticoids (HR 2.0; 1.3-3.0), and swollen joint count >7 (HR 0.36; 0.24-0.55) were independent predictors. Five-year drug survival was 31%, 43%, and 49% for infliximab, adalimumab, and etanercept, respectively (p = 0.010). Infliximab was associated with significantly more withdrawals due to adverse events. Disease activity, CRP, and use of glucocorticoids predicted efficacy-related drug survival; age, use of methotrexate, and prior DMARDs failures predicted safety-related survival. Risk for serious infections was lower with adalimumab (odds ratio [OR] 0.62; 0.38-1.00) or etanercept (OR 0.39; 0.21-0.72) than infliximab, independent of the effects of age (OR 1.65; 1.37-2.00 per 10 years), tender joint count >10 (OR 1.86; 1.21-2.86), and glucocorticoids >35mg/week (OR 1.83; 1.12-2.99)., Conclusions: Response rates were comparable among anti-TNF agents. Overall, 5-year drug survival was below 50%, with infliximab demonstrating increased safety-related discontinuations. Remission rates are low in clinical practice. Strategies to increase effectiveness and long-term survival of anti-TNF agents in RA are needed., (Copyright © 2014 Elsevier Inc. All rights reserved.)
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- 2014
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16. Intravenous immunoglobulin treatment for pregnancy-associated dermatomyositis.
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Linardaki G, Cherouvim E, Goni G, and Boki KA
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- Adult, Female, Humans, Pregnancy, Treatment Outcome, Dermatomyositis therapy, Immunoglobulins, Intravenous therapeutic use, Pregnancy Complications therapy
- Abstract
Pregnancy-associated dermatomyositis (DM) is a rare disorder, until recently treated only with corticosteroids due to the toxicity of other immunosuppressive agents for the fetus. We present a pregnant woman with DM treated successfully with intravenous immunoglobulin (IVIG) and medium dose corticosteroids. A 42-year-old woman presented with a rash, muscle weakness and increased muscle enzymes on the 15th week of her first pregnancy. After the diagnosis of DM she was treated with the combination of medium dose corticosteroids and IVIG. The patients' symptoms resolved rapidly. No complications were noted for either her or the fetus. Both she and her son remain disease-free after 6 years follow-up. In conclusion, IVIG treatment is a safe and effective alternative for pregnancy-associated DM.
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- 2011
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17. Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis.
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Tzelepis GE, Kokosi M, Tzioufas A, Toya SP, Boki KA, Zormpala A, and Moutsopoulos HM
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- Antibodies, Antineutrophil Cytoplasmic analysis, Female, Follow-Up Studies, Glomerulonephritis diagnosis, Glomerulonephritis mortality, Hemoptysis diagnosis, Humans, Male, Microscopic Polyangiitis diagnosis, Microscopic Polyangiitis mortality, Middle Aged, Prevalence, Prognosis, Pulmonary Fibrosis diagnostic imaging, Pulmonary Fibrosis mortality, Radiography, Treatment Outcome, Microscopic Polyangiitis epidemiology, Pulmonary Fibrosis epidemiology
- Abstract
We sought to determine the type of pulmonary involvement in microscopic polyangiitis (MPA), primarily focusing on pulmonary fibrosis (PF), its prevalence, temporal relationship with other disease manifestations and outcome. 33 patients (16 males) with biopsy proven perinuclear anti-neutrophilic cytoplasmic antibody-positive MPA (age 63.5 yrs) participated in the study. Pulmonary involvement was assessed using standard methods, including radiographic imaging (chest radiographs and high-resolution computed tomography), pulmonary function testing, bronchoscopy and bronchoalveolar lavage, and, if indicated, lung biopsy. All-cause mortality was analysed by the Kaplan-Meier method and was compared between MPA patients with and without PF. At the time of diagnosis, renal involvement was detected in all patients, with renal biopsies being consistent with segmental necrotising glomerulonephritis in all patients. The most common respiratory symptom was haemoptysis, which was found in nine (27%) patients. PF was present in 12 (36%) patients at the time of diagnosis, whereas one patient developed PF while on therapy approximately 10 yrs after disease diagnosis. In seven patients with PF, respiratory symptoms related to fibrosis preceded other disease manifestations by a median (range) period of 13 (5-120) months. Patients were followed up for a period of 38+/-30 months. Presence of PF was associated with increased mortality (p = 0.02), with six deaths occurring in the fibrotic group and one in the nonfibrotic group. In the fibrotic group most deaths were related to PF. PF occurs frequently in MPA, may precede other disease manifestations by a variable length of time and has a poor prognosis.
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- 2010
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18. Thoracoabdominal motion in ankylosing spondylitis: association with standardised clinical measures and response to therapy.
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Tzelepis GE, Kalliakosta G, Tzioufas AG, Sfikakis PP, Mandros C, Boki KA, Roussos C, and Moutsopoulos HM
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- Abdomen physiopathology, Adult, Analysis of Variance, Antirheumatic Agents therapeutic use, Case-Control Studies, Female, Humans, Male, Middle Aged, Plethysmography, Impedance, Posture, Respiration, Spondylitis, Ankylosing drug therapy, Treatment Outcome, Tumor Necrosis Factor-alpha antagonists & inhibitors, Movement, Spondylitis, Ankylosing physiopathology
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Objectives: To assess the relationship between thoracoabdominal motion during quiet breathing and standardised indices of disease severity in patients with ankylosing spondylitis (AS); also to evaluate whether thoracoabdominal motion improves after institution of biological agents in these patients., Methods: Displacement of the rib cage (RC) and abdomen (Abd) during quiet breathing in the sitting, standing and supine position were recorded by impedance plethysmography in 60 patients (mean (SD) age 41 (10) years, 56 men) and 21 healthy men (mean (SD) 36 (7) years). x-y plots of RC versus Abd displacement during quiet breathing were constructed, and the angle of the slope of the RC-Abd loop was calculated and averaged for five consecutive breaths. In 13 patients treated with anti-tumour necrosis factor alpha (TNFalpha), measurements were made before and at 3, 6 and 12 months after the start of treatment., Results: In the entire AS group, the angle of the slope of the RC-Abd loop correlated with Bath Ankylosing Spondylitis Functional Index (BASFI) in the sitting (R = -0.50, p<0.0001), standing (R = -0.36, p = 0.004) and supine (R = -0.47, p = 0.0001) position, but not with Bath Ankylosing Spondylitis Disease Activity (BASDAI), Bath Ankylosing Spondylitis Metrology Index (BASMI) or the modified Schober's test. In 13 patients treated with anti-TNFalpha, the angle of the RC-Abd slope improved significantly (35-69% over baseline at 3 months) in all body positions and in a nearly parallel fashion with the improvements in standardised clinical measurements., Conclusions: The pattern of thoracoabdominal motion during quiet breathing correlates with BASFI, and its response to anti-TNFalpha treatment is large. This variable may be an appropriate target for evaluating potential usefulness in monitoring thoracic spine involvement and response to treatment in AS.
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- 2009
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19. Adalimumab is effective and well tolerated in treating patients with ankylosing spondylitis who have advanced spinal fusion.
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Rudwaleit M, Olivieri I, Boki KA, Griep EN, Järvinen P, Wong RL, Kron M, Kary S, and Kupper H
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- Adalimumab, Adult, Aged, Antibodies, Monoclonal administration & dosage, Antibodies, Monoclonal adverse effects, Antibodies, Monoclonal, Humanized, Antirheumatic Agents administration & dosage, Antirheumatic Agents adverse effects, Drug Administration Schedule, Female, Humans, Male, Middle Aged, Radiography, Severity of Illness Index, Spondylitis, Ankylosing diagnostic imaging, Spondylitis, Ankylosing pathology, Treatment Outcome, Tumor Necrosis Factor-alpha antagonists & inhibitors, Antibodies, Monoclonal therapeutic use, Antirheumatic Agents therapeutic use, Spondylitis, Ankylosing drug therapy
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Objectives: To evaluate the effectiveness and safety of adalimumab in treating patients with AS and advanced structural damage., Methods: Patients with active AS [Bath AS Disease Activity Index (BASDAI) > or =4] received 40 mg of adalimumab every other week plus their standard anti-rheumatic therapies in this 12-week, open-label study. Investigators documented the presence or absence of advanced ankylosis based on previous radiographs. Stages IV (from 50 to <80% involvement in more than two spinal segments) and V (> or =80% spinal involvement, including bamboo spine) disease were considered as advanced AS. Effectiveness parameters included Assessment of SpondyloArthritis international Society (ASAS) criteria, BASDAI response and achievement of optimal sleep. Adverse events were reported throughout therapy and at a 70-day follow-up., Results: The analysis population included 897 patients whose AS was not advanced (i.e. Stages I-III), 31 with Stage IV disease and 41 with Stage V disease. At Week 12, ASAS40/BASDAI 50 responses were achieved by 54%/57% of patients with AS Stages I-III, 48%/58% with AS Stage IV and 54%/66% with AS Stage V, respectively. ASAS partial remission rates were 30, 26 and 7% for patients with Stages I-III, IV and V disease, respectively. Serious infections occurred in three (<1%) patients with AS Stages I-III and in one (1%) patient with AS Stage V., Conclusions: After 12 weeks of adalimumab therapy, patients with advanced but active AS, including those with structural damage of > or =80% of the vertebrae, achieved improvements in signs and symptoms similar to those attained by patients whose AS was not advanced.
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- 2009
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20. Chest CT findings in patients with inflammatory myopathy and Jo1 antibodies.
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Karadimitrakis S, Plastiras SC, Zormpala A, Chatzikonstantinou K, Boki KA, Tzelepis GE, and Moutsopoulos HM
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- Female, Humans, Lung Diseases, Interstitial diagnostic imaging, Male, Middle Aged, Radiography, Thoracic, Retrospective Studies, Syndrome, Autoantibodies, Lung Diseases diagnostic imaging, Myositis complications, Tomography, X-Ray Computed methods
- Abstract
Thoracic high-resolution computed tomography scans (HRCT) of 17 patients with inflammatory muscle disorders (IMD) and positive Jo1 antibodies were retrospectively reviewed regarding presence, extension, and distribution of pathological findings. Abnormal findings were found in 14 (82.3%) patients. The predominant CT abnormality was ground glass attenuation, which was present in seven patients (41.1%), having a bilateral and diffuse distribution. In general, lesions tended to appear in the lower lobes and more specifically in the lung bases. Interlobular septal thickening was found in six patients (35.3%); it was seen in the upper and lower lobes with peripheral distribution and bilateral localization in five out of six patients. Bronchiectases, reticular opacities, and honeycombing were found in six patients (35.3%). Air space consolidation was seen in about 17% of the patients. Lung involvement is a frequent feature of IMD patients with positive Jo1 antibodies and its most common radiological pattern is that of nonspecific interstitial pneumonia.
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- 2008
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21. Is the heart affected in primary Sjögren's syndrome? An echocardiographic study.
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Vassiliou VA, Moyssakis I, Boki KA, and Moutsopoulos HM
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- Age Factors, Aortic Valve Insufficiency complications, Complement C4 analysis, Cryoglobulinemia complications, Female, Humans, Hypertension, Pulmonary complications, Liver Cirrhosis, Biliary complications, Male, Middle Aged, Mitral Valve Insufficiency complications, Pericardial Effusion complications, Tricuspid Valve Insufficiency complications, Echocardiography, Doppler, Color, Heart physiopathology, Sjogren's Syndrome physiopathology
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Objective: To evaluate whether patients with primary Sjögren's syndrome without overt cardiac disease have echocardiographic abnormalities and their relation with clinical and laboratory data., Methods: One hundred and seven consecutive patients with primary Sjögren's syndrome and 112 healthy controls, matched for age and gender, underwent complete echocardiographic study., Results: Thirty-two patients had mitral valve regurgitation (p<0.001) whereas tricuspid and aortic valve regurgitation were, also, more frequent in the patient group (p=0.022 and p=0.007 respectively). In multivariate analyses, low C4 levels of complement and age were strong predictors of mitral valve regurgitation whereas age was predictor of aortic valve regurgitation. Tricuspid valve regurgitation was associated with pulmonary hypertension. Clinically silent pericardial effusion, found in 9 patients (p=0.008), was associated with cryoglobulinemia and primary biliary cirrhosis. Twenty-four patients had pulmonary hypertension (p<0.001) whereas hypocomplementemia and cryoglobulinemia were strong predictors of pulmonary artery systolic pressure. The analyses reveal that easy fatigability was associated with pulmonary hypertension and low C4 levels. The patients' left ventricular mass index differed significantly from the controls (108.9+/-17.21 gm(-2) vs. 85.8+/-6.73 gm(-2), p<0.001) and was associated with palpaple purpura and anti-Ro/SSA. From the diastolic function indices only the left ventricular isovolumic relaxation time differed significantly among patients and controls., Conclusion: Valvular regurgitation, pericardial effusion, pulmonary hypertension and increased left ventricular mass index occur with disproportionately high frequency in patients with primary Sjögren's syndrome and no clinically apparent heart disease. Thus echocardiographic studies may need to be performed in these patients especially when palpable purpura, antibody reactivity and low C4 levels are present.
- Published
- 2008
22. Lupus nephritis: treatment with mycophenolate mofetil.
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Kapitsinou PP, Boletis JN, Skopouli FN, Boki KA, and Moutsopoulos HM
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- Adult, Drug Therapy, Combination, Female, Glucocorticoids therapeutic use, Humans, Kidney pathology, Lupus Nephritis pathology, Male, Retrospective Studies, Statistics, Nonparametric, Immunosuppressive Agents therapeutic use, Lupus Nephritis drug therapy, Mycophenolic Acid analogs & derivatives, Mycophenolic Acid therapeutic use
- Abstract
Objective: To evaluate the safety and efficacy of mycophenolate mofetil (MMF) treatment in patients with lupus nephritis., Methods: Eighteen patients with biopsy-proven lupus nephritis (17 females, one male; mean age 31.6 yr; mean lupus duration 92 months; mean duration of nephritis 57 months; nine with focal proliferative glomerulonephritis, three with diffuse proliferative glomerulonephritis, six with membranous nephropathy) were included. With five exceptions, all patients had been treated previously with cyclophosphamide and were selected because of either toxicity or inadequate clinical response to treatment. MMF was given at 2 g daily in combination with steroids for up to 31 months (mean 15.3 months). The side-effects of MMF were recorded and efficacy was assessed as the renal function profile., Results: Complete remission was observed in 10/18 patients and another 4/18 went into partial remission. Both creatinine clearance and proteinuria were significantly improved during MMF treatment in patients with the proliferative forms of nephritis. MMF demonstrated a steroid-sparing effect in the whole population. Treatment failure was recorded in 4/18 patients, all with membranous nephropathy. Two patients developed gastrointestinal complaints and infectious meningitis occurred in one patient., Conclusion: MMF appears to be an efficacious and safe treatment in patients with proliferative forms of lupus nephritis who do not respond to or cannot tolerate conventional treatment. The efficacy of MMF in lupus membranous nephropathy remains unclear.
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- 2004
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23. Shared epitopes and rheumatoid arthritis: disease associations in Greece and meta-analysis of Mediterranean European populations.
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Ioannidis JP, Tarassi K, Papadopoulos IA, Voulgari PV, Boki KA, Papasteriades CA, and Drosos AA
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- Adolescent, Adult, Aged, Arthritis, Rheumatoid epidemiology, Arthritis, Rheumatoid physiopathology, Female, Greece epidemiology, Humans, Male, Mediterranean Region epidemiology, Middle Aged, Severity of Illness Index, Arthritis, Rheumatoid genetics, Genetic Predisposition to Disease, Histocompatibility Antigens Class II genetics
- Abstract
Objectives: To assess the strength of the associations between HLA shared epitopes (SE) and rheumatoid arthritis (RA) susceptibility, articular disease severity, and extra-articular features in Mediterranean European populations., Methods: One hundred and seventy-four Greek RA patients and 103 controls were evaluated. Data were then included in a meta-analysis of 9 studies of Mediterranean European populations (959 RA patients and 1,405 controls)., Results: In our study population, SE alleles were significantly more common in RA patients than in controls (odds ratio [OR], 2.5; 95% confidence interval [CI], 1.4-4.3). Larsen radiologic score was predicted by SE and disease duration. SE did not increase the risk of any extra-articular manifestation. The meta-analysis showed a pooled OR of 3.7 (95% CI, 2.6-5.2) for susceptibility to RA conferred by SE (OR, 3.4 v 3.9 in Greek v non-Greek populations)., Conclusions: SE determine articular destruction without increasing the risk of extra-articular manifestations. The immunogenetic associations of RA susceptibility are consistent, but their strength may depend on the SE prevalence in different ethnic groups., (Copyright 2002, Elsevier Science (USA). All rights reserved.)
- Published
- 2002
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24. How significant is sensorineural hearing loss in primary Sjögren's syndrome? An individually matched case-control study.
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Boki KA, Ioannidis JP, Segas JV, Maragkoudakis PV, Petrou D, Adamopoulos GK, and Moutsopoulos HM
- Subjects
- Aged, Auditory Threshold, Case-Control Studies, Electronystagmography, Evoked Potentials, Auditory, Brain Stem, Hearing, Humans, Middle Aged, Vestibule, Labyrinth physiopathology, Hearing Loss, Sensorineural etiology, Hearing Loss, Sensorineural physiopathology, Sjogren's Syndrome complications
- Abstract
Objective: We evaluated whether sensorineural loss and vestibular abnormalities are common in patients with primary Sjögren's syndrome (pSS) and whether such abnormalities are clinically significant., Methods: In an individually matched case-control design, 48 patients with pSS underwent complete audiovestibular evaluation along with 48 age and sex matched individuals without otologic problems. Differences of > 20 dB between patient and control ears at any frequency tested were considered to be significant., Results: Significant differences in hearing loss were seen at 4,000 Hz (6 vs 0 ears; p = 0.03) and at 8,000 Hz (9 vs 0 ears; p = 0.003). Small differences in hearing acuity were also observed in the lower frequencies, but the absolute mean difference was < 3 dB. A decrease of at least 60 dB in hearing acuity at any frequency up to 4,000 Hz was seen only in 3 elderly pSS patients. Abnormal brainstem auditory evoked responses were recorded in 7 patients and 5 controls, but no patient had retrocochlear lesions identified on magnetic resonance imaging. Four patients in each group had abnormalities on electronystagmography., Conclusion: pSS is associated with sensorineural hearing loss affecting preferentially the high frequencies, but clinically significant defects are not common. There is no evidence of retrocochlear disease or increased vestibular involvement in pSS.
- Published
- 2001
25. Right ventricular diastolic dysfunction in patients with anticardiolipin antibodies and antiphospholipid syndrome.
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Tektonidou MG, Ioannidis JP, Moyssakis I, Boki KA, Vassiliou V, Vlachoyiannopoulos PG, Kyriakidis MK, and Moutsopoulos HM
- Subjects
- Adult, Antiphospholipid Syndrome immunology, Biomarkers blood, Cross-Sectional Studies, Diastole physiology, Echocardiography, Doppler, Color, Echocardiography, Doppler, Pulsed, Female, Humans, Immunoglobulin G blood, Lupus Erythematosus, Systemic complications, Male, Regression Analysis, Ventricular Dysfunction, Right diagnostic imaging, Ventricular Dysfunction, Right immunology, Antibodies, Anticardiolipin blood, Antiphospholipid Syndrome complications, Ventricular Dysfunction, Right etiology
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Objective: To evaluate the prevalence of diastolic dysfunction in patients with anticardiolipin antibodies (aCL) and to examine whether the antiphospholipid syndrome (APS) is associated with diastolic dysfunction independently of valvular abnormalities and systolic dysfunction., Methods: Pulsed, continuous, colour Doppler echocardiography was performed in 179 subjects, of whom 15 were excluded from the analysis because of systolic dysfunction or severe valvular disease. The remaining 164 subjects included 29 patients with primary APS, 26 patients with secondary APS (APS in the presence of systemic lupus erythematosus (SLE)), and 30 patients with SLE and aCL but without APS; 43 patients with SLE without aCL and 36 normal volunteers served as control groups., Results: The groups compared differed significantly in all measures of right ventricular function. There was a gradation of increasing diastolic function impairment as manifested by prolonged deceleration time (DT) and isovolumic relaxation time (IVRT) across the groups of patients with SLE without aCL, SLE with aCL, secondary APS, and primary APS. Differences in left ventricular diastolic function measures were less prominent. In regression analysis, DT increased by 19.6 ms (p=0.002) in the presence of primary APS and by 20.1 ms (p=0.038) in the presence of pulmonary hypertension. The titre of IgG aCL was the strongest predictor of a prolonged IVRT., Conclusion: Diastolic dysfunction, in particular of the right ventricle-that is, independent of valvular disease and systolic dysfunction, is a prominent feature of APS and may be related to the pathogenesis of the syndrome.
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- 2001
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26. Predictors of clinical outcome and radiologic progression in patients with neuropsychiatric manifestations of systemic lupus erythematosus.
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Karassa FB, Ioannidis JP, Boki KA, Touloumi G, Argyropoulou MI, Strigaris KA, and Moutsopoulos HM
- Subjects
- Adult, Antiphospholipid Syndrome diagnostic imaging, Antiphospholipid Syndrome immunology, Antiphospholipid Syndrome pathology, Brain diagnostic imaging, Disease Progression, Female, Follow-Up Studies, Humans, Lupus Erythematosus, Systemic complications, Lupus Erythematosus, Systemic diagnostic imaging, Lupus Erythematosus, Systemic immunology, Lupus Erythematosus, Systemic pathology, Lupus Erythematosus, Systemic therapy, Magnetic Resonance Imaging, Male, Middle Aged, Predictive Value of Tests, Prognosis, Prospective Studies, Radiography, Treatment Outcome, Antiphospholipid Syndrome complications, Brain pathology, Lupus Erythematosus, Systemic diagnosis
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Purpose: We sought to identify the predictors of clinical outcome and of the evolution of cerebral abnormalities in patients with neuropsychiatric systemic lupus erythematosus (SLE)., Subjects and Methods: Thirty-two patients with SLE (including 14 with the antiphospholipid syndrome) who had been hospitalized with primary neuropsychiatric disease were observed prospectively for at least 2 years. Laboratory and clinical characteristics and data from magnetic resonance imaging (MRI) studies obtained during the hospitalization and 2 years later were evaluated. We ascertained nonreversible or new MRI changes and clinical outcomes, including neuropsychiatric events, during follow-up., Results: Cranial MRI scans on admission were abnormal in 26 (81%) of the 32 patients. Patients with the antiphospholipid syndrome were more likely to have focal cerebral white matter lesions (odds ratio [OR] = 12, 95% confidence interval [CI]: 2.0 to 72). After 2 years, neuropsychiatric deficits substantially improved in 22 (69%) of the patients, stabilized in 6 (19%), and deteriorated in 4 (12%). The number of prior neuropsychiatric events was associated with persistent MRI lesions (OR = 4.8 per each event, 95% CI: 1.1 to 21) and unfavorable clinical outcome (OR = 4.3 per each event, 95% CI: 1.4 to 13) at 2 years. The antiphospholipid syndrome also predicted an unfavorable clinical outcome at 2 years (OR = 11, 95% CI: 1.7 to 65)., Conclusions: Among patients with SLE who have neuropsychiatric disease, prior neuropsychiatric events and the antiphospholipid syndrome increase the risk of adverse outcomes.
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- 2000
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27. Prognostic factors and clustering of serious clinical outcomes in antiphospholipid syndrome.
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Tektonidou MG, Ioannidis JP, Boki KA, Vlachoyiannopoulos PG, and Moutsopoulos HM
- Subjects
- Adult, Antibodies, Anticardiolipin analysis, Anticoagulants adverse effects, Anticoagulants therapeutic use, Antiphospholipid Syndrome drug therapy, Antiphospholipid Syndrome immunology, Aspirin adverse effects, Autoantibodies analysis, Cluster Analysis, Disease Progression, Enzyme-Linked Immunosorbent Assay, Female, Glycoproteins analysis, Glycoproteins immunology, Humans, Immunoglobulin G immunology, Immunoglobulin M immunology, Male, Prognosis, Proportional Hazards Models, Prospective Studies, Retrospective Studies, Severity of Illness Index, Time Factors, Warfarin adverse effects, Antiphospholipid Syndrome complications
- Abstract
We assessed whether initial clinical presentations suggestive of antiphospholipid syndrome (APS) predicted the subsequent rate and type of serious clinical outcomes. Eighty-two consecutive patients with anticardiolipin antibodies or lupus anticoagulant were followed for 814 person-years after a first event suggestive of APS (livedo reticularis, thrombocytopenia, autoimmune haemolysis, thrombosis, central nervous system manifestations, recurrent abortions). The hazard of developing a second event was largest in patients with antibodies recognizing beta2 glycoprotein I who had autoimmune haemolysis as the first event (hazard ratio HR 2.70, p=0.018) and smallest in patients without such antibodies who had recurrent abortions as their first event (HR 0.37, p=0.028). Subsequent serious events in patients with venous and arterial thromboses, recurrent abortions, central nervous system manifestations and autoimmune haemolytic anaemia were likely to be of the same type as the presenting event (odds ratio (OR) 3.76, 5.90, 77.7, 6.92, and 7.13, respectively. Adjusting for therapy, the rate of subsequent serious events was 6.86-fold higher (p=0.0001) in patients presenting with two events, 1.56-fold higher (p=0.038) in autoimmune haemolysis presentations, 1.69-fold higher (p=0.004) in patients with anti-beta2-glycoprotein-I antibodies, and 46% (p=0.063) lower in thrombocytopenia presentations. Initial clinical features determine the long-term evolution of APS, and specific types of clinical manifestations cluster during the course of the disease.
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- 2000
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28. Risk factors for central nervous system involvement in systemic lupus erythematosus.
- Author
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Karassa FB, Ioannidis JP, Touloumi G, Boki KA, and Moutsopoulos HM
- Subjects
- Adolescent, Adult, Aged, Antibodies, Anticardiolipin analysis, Antibodies, Antinuclear immunology, Case-Control Studies, Child, Child, Preschool, Enzyme-Linked Immunosorbent Assay, Female, Fluorescent Antibody Technique, Humans, Immunoglobulin G analysis, Male, Middle Aged, Pregnancy, Risk Factors, Lupus Vasculitis, Central Nervous System etiology
- Abstract
We investigated risk factors for central nervous system (CNS) involvement in systemic lupus erythematosus (SLE), in 32 such patients individually matched 1 : 3 to 96 control SLE patients without CNS events. Univariate analysis showed that CNS involvement was significantly associated with the antiphospholipid syndrome (APS) as well as its features: arterial thrombosis, recurrent fetal loss, livedo reticularis and IgG anticardiolipin (aCL) antibodies in high titres. Other potential associations included cutaneous vasculitic lesions, thrombocytopenia, positive ANA, anti-SS-B/La and low serum levels of C(3) and C(4) complement components, while articular manifestations and discoid rash were significantly less common in patients with neuropsychiatric (NP) disease. In multivariate modeling, CNS involvement was strongly associated with cutaneous vasculitic lesions OR 33, 95% CI 1.5-720) and arterial thromboses (OR 13, 95%CI 0.82-220), and negatively related to the presence of articular manifestations (OR 0.015, 95%CI 0.00-0.17) and discoid rash (OR 0.004, 95%CI 0.00-0.35). Associations with APS-related arterial thromboses and vasculitis point to the importance of arterial vascular pathophysiology in the pathogenesis of NP disease in SLE. Patients with articular manifestations and discoid rash are at very low risk of NP events. Patients with an adverse SLE disease profile may require closer observation and may be the target group for studying pre-emptive interventions.
- Published
- 2000
- Full Text
- View/download PDF
29. The clinical relevance of antibodies to ribosomal-P common epitope in two targeted systemic lupus erythematosus populations: a large cohort of consecutive patients and patients with active central nervous system disease.
- Author
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Tzioufas AG, Tzortzakis NG, Panou-Pomonis E, Boki KA, Sakarellos-Daitsiotis M, Sakarellos C, and Moutsopoulos HM
- Subjects
- Adult, Animals, Biomarkers blood, Case-Control Studies, Cohort Studies, Enzyme-Linked Immunosorbent Assay methods, Epitopes immunology, Female, Humans, Lupus Vasculitis, Central Nervous System diagnosis, Lupus Vasculitis, Central Nervous System immunology, Male, Rats, Sensitivity and Specificity, Autoantibodies blood, Autoimmune Diseases immunology, Lupus Erythematosus, Systemic immunology, Protozoan Proteins, Ribosomal Proteins immunology
- Abstract
Objectives: To develop an enzyme linked immunosorbent assay (ELISA) using as substrate a synthetic 22-aminoacid peptide, corresponding to the ribosomal P0, P1 and P2 common epitope. To study the specificity and sensitivity of the method and evaluate the frequency and clinical associations of anti-P antibodies in two groups of systemic lupus erythematosus (SLE) patients: (a) unselected SLE patients and (b) SLE patients with central nervous system (CNS) involvement., Patients and Methods: The C-terminal 22 aminoacid peptide of the ribosomal P proteins (Lys-Lys-Glu-Glu-Lys-Lys-Glu-Glu-Lys-Ser-Glu-Glu-Glu-Asp-Glu-Asp-Met- Gly-Phe-Gly-Leu-Phe-Asp) was synthesised according to Merrifield's solid phase procedure. Purification of the peptide was performed by preparative high performance liquid chromatography and confirmed by amino acid analysis. Using this peptide, in a concentration 5 microg/ml, an ELISA was developed. The presence of anti-P antibodies was evaluated by western blot using purified ribosomal proteins from rat liver. Sera from 178 consecutive patients with SLE and 28 patients with SLE and CNS manifestations were tested. Sera from 58 patients with rheumatoid arthritis and 57 patients with primary Sjögren's syndrome were used as controls. The cut off point of the assay was defined using 124 normal sera., Results: The specificity of the assay was evaluated by homologous inhibition. Pretreatment of positive sera with soluble 22mer peptide of the ribosomal P proteins resulted in 88% inhibition. The concordance between the peptide assay and western blot was found to be 83%. Thirty three of 178 (18. 6%) of the unselected SLE patients had antibodies to P-protein common epitope. Their presence was associated with more active disease (European Consensus Lupus Activity Measurement, ECLAM scoring system) (p<0.001), higher levels of anti-ds DNA antibodies (p<0.05) and lower levels of the C4 component of complement (p<0.01). Eleven of 28 (39.3%) patients with SLE and active CNS involvement had antibodies to P-protein. The overall prevalence of anti-P antibodies in active CNS disease patients was statistically significantly higher, as compared with unselected SLE patients (chi(2)=6.04, p<0.05). These antibodies were found in a high proportion of patients without anticardiolipin antibodies (52.4%) and they were associated with diffuse CNS involvement (psychiatric disorders (71%) and epilepsy (75%))., Conclusions: A synthetic analogue of the common epitope of ribosomal P-proteins can be use as an antigen for the detection of anti-P antibodies. These antibodies are associated with active SLE and CNS involvement particularly in patients without anticardiolipin antibodies.
- Published
- 2000
- Full Text
- View/download PDF
30. Remission, relapse, and re-remission of proliferative lupus nephritis treated with cyclophosphamide.
- Author
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Ioannidis JP, Boki KA, Katsorida ME, Drosos AA, Skopouli FN, Boletis JN, and Moutsopoulos HM
- Subjects
- Cohort Studies, Humans, Recurrence, Remission Induction, Cyclophosphamide therapeutic use, Lupus Nephritis drug therapy
- Abstract
Unlabelled: Remission, relapse, and re-remission of proliferative lupus nephritis treated with cyclophosphamide., Background: Long-term intravenous cyclophosphamide (IVC) in combination with corticosteroids is standard therapy for proliferative lupus nephritis, but it has limitations. There are few data on long-term remission rates, predictors of relapse, and the ability to achieve a second remission with currently recommended IVC regimens., Methods: A cohort of 85 patients with proliferative lupus glomerulonephritis (focal N = 33, diffuse N = 52) treated with IVC was assembled in three institutions. Timing and predictors of remission, relapse, and re-remission were evaluated with Kaplan-Meier analyses and Cox models., Results: The median time to remission was 10 months, whereas an estimated 22% of patients had not remitted after 2 years. The median time to relapse among 63 patients who had achieved remission was 79 months. In multivariate models, adverse predictors of remission were a delay in the initiation of therapy from the time nephritis was clinically diagnosed [hazard ratio (HR) 0.58, P = 0. 063] and a higher amount of proteinuria (HR 0.86 per 1 g/24 hours, P = 0.014). Predictors of earlier relapse for patients entering remission included a longer time to remission (HR 1.029 per month, P = 0.025), a history of central nervous system involvement (HR 8.41, P = 0.002), and World Health Organization histology (P = 0.01). Among the 23 patients who relapsed during follow-up, the median time to re-remission was 32 months, and with three exceptions, all patients took substantially longer time to remit the second time compared with their first remission (P = 0.01). The time to re-remission was longer in patients who had taken longer to remit the first time (HR 0.979 per month, P = 0.16), in patients who had relapsed earlier after the first remission (HR 1.071 per month, P = 0.002), and in those with evidence of chronicity in the original kidney biopsy (P = 0.015)., Conclusions: Prolonged courses with a cumulative risk of toxicity are needed to achieve remission in many first-treated patients and in most patients treated for a second time. The optimal management of patients with identified adverse predictors of response needs further study.
- Published
- 2000
- Full Text
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31. Takayasu's arteritis-associated aneurysm formation without steno-occlusive lesions.
- Author
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Boki KA, Pakas J, Kekatou K, Kataxaki E, and Papapavlou E
- Subjects
- Adult, Female, Humans, Aortic Aneurysm diagnostic imaging, Aortic Aneurysm etiology, Aortography, Radiography, Thoracic, Takayasu Arteritis complications, Tomography, X-Ray Computed
- Abstract
The authors report a case of a 20-year-old woman with Takayasu's arteritis (TA) presenting with recurrent erythema nodosum-like lesions, elevated acute-phase proteins and aortographic findings of multiple aneurysmal dilatations of the aorta without the coexistence of steno-occlusive lesions. This finding indicates that aneurysms could be an early manifestation of TA and not necessarily a change secondary to stenotic lesions.
- Published
- 2000
- Full Text
- View/download PDF
32. Intravenous immunoglobulin compared with cyclophosphamide for proliferative lupus nephritis.
- Author
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Boletis JN, Ioannidis JP, Boki KA, and Moutsopoulos HM
- Subjects
- Adolescent, Adult, Drug Administration Schedule, Female, Humans, Injections, Intravenous, Lupus Erythematosus, Systemic drug therapy, Male, Middle Aged, Pilot Projects, Treatment Outcome, Cyclophosphamide therapeutic use, Immunoglobulins, Intravenous therapeutic use, Immunosuppressive Agents therapeutic use, Lupus Nephritis drug therapy
- Abstract
Among 14 randomised patients with proliferative lupus nephritis, monthly intravenous immunoglobulin maintained remission over 18 months, similar to standard intravenous cyclophosphamide treatment. Pulsed immunoglobulin may be a useful alternative therapy in lupus nephritis.
- Published
- 1999
- Full Text
- View/download PDF
33. Pure red cell aplasia as presentation of systemic lupus erythematosus: antibodies to erythropoietin.
- Author
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Linardaki GD, Boki KA, Fertakis A, and Tzioufas AG
- Subjects
- Adult, Antibodies blood, Diagnosis, Differential, Enzyme-Linked Immunosorbent Assay, Erythropoietin immunology, Female, Humans, Lupus Erythematosus, Systemic complications, Red-Cell Aplasia, Pure complications, Lupus Erythematosus, Systemic diagnosis, Red-Cell Aplasia, Pure diagnosis
- Abstract
In this case report we describe two patients with pure red cell aplasia (PRCA) as an initial manifestation of systemic lupus erythematosus (SLE). Antibodies to erythropoietin were determined, by an ELISA method developed in our laboratory, in frozen serum obtained from one of the patients. A high titer of antibodies to erythropoietin was detected in serum obtained before treatment with high dose intravenous immunoglobulin (IVIG). The antibody titer declined after successful treatment. This observation suggests that antibodies to erythropoietin may contribute to the pathogenesis of SLE associated PRCA.
- Published
- 1999
- Full Text
- View/download PDF
34. Necrotizing vasculitis in Greece: clinical, immunological and immunogenetic aspects. A study of 66 patients.
- Author
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Boki KA, Dafni U, Karpouzas GA, Papasteriades C, Drosos AA, and Moutsopoulos HM
- Subjects
- Adolescent, Adult, Aged, Antibodies, Antineutrophil Cytoplasmic analysis, Antirheumatic Agents therapeutic use, Autoimmune Diseases epidemiology, Autoimmune Diseases genetics, Autoimmune Diseases immunology, Churg-Strauss Syndrome epidemiology, Churg-Strauss Syndrome genetics, Churg-Strauss Syndrome immunology, Cyclophosphamide therapeutic use, Disease Progression, Female, Granulomatosis with Polyangiitis epidemiology, Granulomatosis with Polyangiitis genetics, Granulomatosis with Polyangiitis immunology, Greece epidemiology, HLA Antigens analysis, HLA Antigens genetics, HLA Antigens immunology, Hepatitis B Surface Antigens analysis, Humans, Incidence, Male, Middle Aged, Necrosis, Polyarteritis Nodosa epidemiology, Polyarteritis Nodosa genetics, Polyarteritis Nodosa immunology, Retrospective Studies, Survival Rate, Treatment Outcome, Vasculitis epidemiology, Vasculitis genetics, Weight Loss physiology, Vasculitis immunology
- Abstract
The clinical spectrum and outcome of necrotizing vasculitis were evaluated in a retrospective study in order to assess: (1) the clinical expression and evolution of the disease; (2) the response to several therapeutic approaches based on major events (organ involvement causing disability or death); (3) the immunogenetic background of patients. Sixty-six Greek patients fulfilling the ACR criteria for the diagnosis of vasculitis entered the study. Thirty-seven were diagnosed with Wegener's granulomatosis (WG), 22 with polyarteritis nodosa (PAN) and seven with Churg-Strauss syndrome (CSS). The demographic characteristics of patients with WG and PAN were similar. Cutaneous manifestations, gastrointestinal and peripheral nervous system involvement occurred more often in patients with PAN, whereas pulmonary and upper respiratory tract involvement, renal, ear abnormalities and fever were more frequent in patients with WG. Muscle weakness and asthma were found exclusively in patients with PAN and CSS, respectively, while the presence of classic-antineutrophil cytoplasmic antibodies (c-ANCA) characterized WG patients. Hepatitis B surface antigen (HBsAg) was found in 22% of PAN patients. No significant differences were detected when comparing the PAN and WG groups with respect to the first major event (log-rank P = 0.50) with and without potential confounders (age, gender, therapy or c-ANCA). For WG patients, a statistically significant difference was found on different routes of administration of cyclophosphamide (oral vs pulse) (P = 0.006). Regarding the HLA antigens, an increased frequency of DR1 (26.9% vs 10.3%, P = 0.057) in WG and the absence of DR3 in patients with PAN and CSS were noted. It appears that although the immunogenetic background and the clinical expression of the diseases differ, the response to treatment as well as the evolution and the survival rate of these patients are similar in the two groups.
- Published
- 1997
- Full Text
- View/download PDF
35. Facial heliotrope rash as the initial manifestation of acute myelomonocytic leukemia.
- Author
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Tzavara V, Stamoulis K, Aroni KG, Kordossis T, and Boki KA
- Subjects
- Aged, Antimetabolites, Antineoplastic therapeutic use, Antineoplastic Combined Chemotherapy Protocols, Arthritis complications, Bone Marrow pathology, Cytarabine therapeutic use, Dermatitis drug therapy, Face pathology, Granulocyte Colony-Stimulating Factor therapeutic use, Humans, Keratosis, Male, Mitoxantrone therapeutic use, Pancytopenia complications, Pancytopenia drug therapy, Parotid Gland pathology, Prednisolone therapeutic use, Dermatitis complications, Leukemia, Myelomonocytic, Acute complications, Leukemia, Myelomonocytic, Acute drug therapy
- Abstract
The association of leukocytoclastic vasculitis or dermatomyositis with malignancies has been reported. We describe a patient who developed a skin rash, histologically compatible with dermatomyositis, which during the course of the disease switched to leukocytoclastic vasculitis, which was accompanied with peripheral blood pancytopenia in the absence of any specific pathological manifestation from the bone marrow three years prior to the diagnosis of acute myelomonocytic leukemia (AMML).
- Published
- 1997
- Full Text
- View/download PDF
36. Mixed cryoglobulinemia in Greece. Primary disorders in 10 cases.
- Author
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Boki KA, Linardaki GD, Galanopoulou V, Vaiopoulos G, Tzioufas AG, and Moutsopoulos HM
- Subjects
- Adult, Aged, Cryoglobulinemia complications, Dizziness complications, Female, Greece epidemiology, Humans, Male, Middle Aged, Purpura complications, Raynaud Disease complications, Weight Loss, Cryoglobulinemia epidemiology
- Abstract
Mixed cryoglobulinemia (MC) is a systemic disorder whose pathogenesis is based on the presence of serum cryoglobulins. The purpose of this study was to evaluate on a prospective basis patients presenting with MC with regard to the clinical manifestations and the underlying disorders. We present ten patients with MC, who were diagnosed and followed up during a one year period in the Division of autoimmune rheumatic diseases (Clinical Department of Pathophysiology). MC was associated with hepatitis C virus (HCV) infection in two cases, with hepatitis B virus (HBV) infection in six, one patient had both HCV and evidence of HBV infection, while the remaining three patients fulfilled European classification criteria for diagnosis of Sjögren's syndrome (SS). In all ten cases, the presence of an underlying factor was identified, being either viral or autoimmune. It is concluded therefore that all patients presenting with MC should be completely evaluated for a hepatitis virus infection or an autoimmune or lymphoproliferative disorder. Furthermore, since the initiation of the process of MC is triggered by many factors, research should be directed towards the identification of the underlying common denominator.
- Published
- 1997
- Full Text
- View/download PDF
37. Thrombotic thrombocytopenic purpura in adult Still's disease.
- Author
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Boki KA, Tsirantonaki MJ, Markakis K, and Moutsopoulos HM
- Subjects
- Adult, Blood Transfusion, Female, Humans, Purpura, Thrombotic Thrombocytopenic therapy, Steroids therapeutic use, Purpura, Thrombotic Thrombocytopenic complications, Still's Disease, Adult-Onset complications
- Abstract
We describe 2 patients with adult Still's disease who developed thrombotic thrombocytopenic purpura (TTP) and were successfully treated. Although TTP has been associated with autoimmune diseases, usually with systemic lupus erythematosus or various forms of vasculitis, it has rarely been observed in patients with adult Still's disease. This uncommon coexistence of 2 clinical entities may indicate similar pathogenetic mechanisms.
- Published
- 1996
38. Giant cell arteritis presenting as pulseless disease of the upper extremities.
- Author
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Hatzis GS, Aroni KG, Kelekis DA, and Boki KA
- Subjects
- Angiography, Anti-Inflammatory Agents therapeutic use, Arm, Diagnosis, Differential, Female, Giant Cell Arteritis drug therapy, Giant Cell Arteritis physiopathology, Humans, Middle Aged, Prednisolone therapeutic use, Takayasu Arteritis drug therapy, Takayasu Arteritis physiopathology, Giant Cell Arteritis diagnosis, Takayasu Arteritis diagnosis
- Abstract
The most frequently recognized clinical features of giant cell arteritis (GCA) derive from the involvement of the cranial arteries. In 10% of patients, however, the aorta and its major branches, are also affected. We report a case of a 53-year-old woman presenting with a fainting episode and diminished pulses in the upper extremities. Histologic examination of the temporal artery revealed features of giant cell arteritis.
- Published
- 1996
- Full Text
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39. Prevalence of antikeratin antibodies in Greek patients with rheumatoid arthritis. A clinical, serologic, and immunogenetic study.
- Author
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Boki KA, Kurki P, Holthofer H, Tzioufas AG, Drosos AA, and Moutsopoulos HM
- Subjects
- Adult, Aged, Arthritis, Rheumatoid ethnology, Female, Fluorescent Antibody Technique, Indirect, Greece, HLA-DR1 Antigen analysis, Humans, Male, Middle Aged, Antibodies analysis, Arthritis, Rheumatoid immunology, Keratins immunology
- Abstract
Objective: The presence of antikeratin antibodies (AKA) has been associated with rheumatoid arthritis (RA) in patients from north and central Europe. Our aim was to investigate the prevalence of AKA in Greek patients with RA., Methods: One hundred and twenty two sera of Greek patients with RA were tested for the presence of AKA by an indirect immunofluorescence technique, and HLA-DR typing was performed by restriction fragment length polymorphism., Results: Nineteen of 122 (16%) Greek patients with RA were positive for AKA. The percentage of AKA in Greek patients with RA is lower than described previously. These antibodies correlated with a male preponderance (p < 0.01) and were associated with the presence of rheumatoid factor (RF) (p < 0.05) and with HLA-DR1 antigen (p < 0.05)., Conclusion: Our results suggest that AKA are present frequently in Greek patients with RA. Their presence was found to be associated with RF and HLA-DR1 antigen. This emphasizes the different clinical expression of RA in Greece.
- Published
- 1995
40. Polymyositis associated with primary biliary cirrhosis.
- Author
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Boki KA and Dourakis SP
- Subjects
- Electromyography, Enzymes blood, Female, Humans, Liver Cirrhosis, Biliary diagnosis, Middle Aged, Muscles enzymology, Muscles pathology, Muscles physiopathology, Polymyositis diagnosis, Polymyositis physiopathology, Liver Cirrhosis, Biliary complications, Polymyositis complications
- Abstract
The coexistence of polymyositis (PM) and primary biliary cirrhosis (PBC) is rare; only nine cases have been described in English literature. We report a case of a 46-year-old woman presenting with these two autoimmune diseases. The diagnosis of PM was based on the symmetrical, proximal limb muscle weakness, elevated muscle enzymes and was confirmed with the electromyography and muscle biopsy. The diagnosis of PBC was based on the increased serum levels of alkaline phosphatase, gamma glutamyltransferase, IgM immunoglobulin, the presence of antimitochondrial antibodies and diagnostic liver biopsy.
- Published
- 1995
- Full Text
- View/download PDF
41. Examination of HLA-DR4 as a severity marker for rheumatoid arthritis in Greek patients.
- Author
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Boki KA, Drosos AA, Tzioufas AG, Lanchbury JS, Panayi GS, and Moutsopoulos HM
- Subjects
- Arthritis, Rheumatoid diagnostic imaging, Arthritis, Rheumatoid immunology, Biomarkers analysis, Female, Greece epidemiology, Hand diagnostic imaging, Humans, Male, Middle Aged, Polymerase Chain Reaction, Polymorphism, Restriction Fragment Length, Radiography, Severity of Illness Index, Arthritis, Rheumatoid ethnology, HLA-DR4 Antigen analysis
- Abstract
Objectives: Previous reports have shown that HLA-DR4 may be a severity marker for rheumatoid arthritis (RA) in patients of northern European origin. The aim of the present study was to investigate this relation in Greek patients with RA, as RA in Greece differs from the RA described previously on clinical, serological, and immunological grounds., Methods: Eighty four patients were studied in whom HLA-DR typing was performed by restriction fragment length polymorphism and the subtypes of HLA-DR4 were determined by the polymerase chain reaction. The absence or presence of HLA-DR4 and its subtypes was correlated with the clinical and serological characteristics of the patients and with the side effects due to disease modifying drugs., Results: Twenty one of the 84 (25%) patients with RA were DR4+. There was no difference between the DR4+ and DR4-patients with respect to duration of disease, severity of arthritis, functional grade, and joint erosion score. The DR4+ group were more likely to have side effects due to disease modifying drugs (43%) than DR4- patients (36%), but this difference was not statistically significant. DR4-patients had more extra-articular manifestations, including Sjögren's syndrome (47 v 19%). Analysis of the DR4 subtypes showed that Dw15 was the most common variant (9/21 patients; 43%). There was no statistical difference in the clinical manifestations among patients with different DR4 subtypes. The same was also true when the clinical picture was correlated with the 'shared RA epitope' (QKRAA/QRRAA/RRRAA), which is common to all HLA-DRB1 alleles positively associated with RA., Conclusions: These results suggest that HLA-DR4 is not a severity marker in Greek patients with RA and further indicate differences in the clinical expression of RA in Greece.
- Published
- 1993
- Full Text
- View/download PDF
42. HLA class II sequence polymorphisms and susceptibility to rheumatoid arthritis in Greeks. The HLA-DR beta shared-epitope hypothesis accounts for the disease in only a minority of Greek patients.
- Author
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Boki KA, Panayi GS, Vaughan RW, Drosos AA, Moutsopoulos HM, and Lanchbury JS
- Subjects
- Amino Acid Sequence, DNA analysis, Female, Gene Amplification, Greece, HLA-DRB1 Chains, Humans, Male, Molecular Sequence Data, Oligonucleotide Probes, Polymorphism, Restriction Fragment Length, Protein Multimerization, Arthritis, Rheumatoid ethnology, Arthritis, Rheumatoid immunology, HLA-DR Antigens genetics, Histocompatibility Antigens Class II genetics
- Abstract
Objective: In Northern Europeans, rheumatoid arthritis (RA) is strongly associated with a relatively conserved pentapeptide sequence of HLA-DR beta found notably in the HLA-DR4 subtypes Dw4 and Dw14 and in DR1. A previous serologic study of HLA class II polymorphism in a Greek population with RA failed to show significant associations with any antigen., Methods: We characterized HLA-DRB polymorphisms in Greek patients with RA and in control subjects by restriction fragment length polymorphism analysis. Allelic DRB subtypes were examined by polymerase chain reaction amplification and oligonucleotide hybridization., Results: DNA analysis in the RA patients showed that although individual HLA-DR allelic associations were weak, a relatively conserved HLA-DR beta motif was significantly associated with RA in this population of Greek patients. The third hypervariable region amino acid sequences QRRAA, QKRAA, or RRRAA were found in the HLA-DR beta 1 of 43.5% of the RA patients versus 15.5% of the controls (uncorrected P = 0.00004)., Conclusion: Sequences shown to influence susceptibility to RA in patients in the UK also play a role in patients in Greece. However, 57% of Greek patients lack the putative HLA-DR beta motif, which suggests that considerable immunogenetic heterogeneity underlies disease susceptibility in this population.
- Published
- 1992
- Full Text
- View/download PDF
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