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1.
Curr Rheumatol Rev ; 2024 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-38874048

RESUMO

Cases of systemic lupus erythematosus (SLE) following psoriatic arthritis (PsA) or vice versa are uncommon. Due to the complexity of autoimmune diseases and the rarity of such cases, comprehensive global data on the co-occurrence of these conditions are limited. Moreover, the pathophysiology concerning the coexistence of SLE and PsA has yet to be fully understood. Interestingly, the progression of both diseases appears to be significantly influenced by the key interleukin (IL) 17, particularly IL-17A. Here, we report 7 cases of SLE and PsA coexistence. In 5 of these cases, PsA occurred before the development of SLE, while in the remaining 2 cases, SLE was diagnosed before PsA. The PsA was characterized mainly by peripheral arthritis without any axial involvement, while the manifestations of SLE varied, with 3 developing systematic severe manifestations. Therapeutic challenges were posed in all cases, as treating one condition could worsen the other. Finally, we review the literature providing the current knowledge on the coexistence of these conditions. Overall, all reported cases emphasize the importance of personalized treatment and careful monitoring for patients with both SLE and PsA.

2.
Front Immunol ; 15: 1342668, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38348033

RESUMO

Objective: To assess the impact conferred by NOD2 variants on the clinical spectrum of patients with systemic autoinflammatory diseases (SAIDs) in Greece. Methods: Consecutive patients (n=167) with confirmed SAIDs who underwent screening by next generation sequencing (NGS) targeting 26 SAID-associated genes, and carried at least one NOD2 gene variant, were retrospectively studied. The demographic, clinical and laboratory parameters were recorded. Results: In total, 24 rare NOD2 variants in 23/167 patients (14%) were detected. Notably, 18 patients had at least one co-existing variant in 13 genes other than NOD2. Nine patients had juvenile- and 14 adult-onset disease. All patients presented with symptoms potentially induced by the NOD2 variants. In particular, the candidate clinical diagnosis was Yao syndrome (YAOS) in 12 patients (7% of the whole SAID cohort). The clinical spectrum of patients with YAOS (mean episode duration 8 days) was fever (n=12/12), articular symptoms (n=8), gastrointestinal symptoms (n=7; abdominal pain/bloating in 7; diarrhea in 4; oral ulcers in 3), serositis (n=7), and rash (n=5), while the inflammatory markers were elevated in all but one patient. Most of these patients showed a poor response to nonsteroidal anti-inflammatory drugs (n=7/9), colchicine (n=6/8) and/or anti-TNF treatment (n=3/4), while a complete response was observed in 6/10 patients receiving steroids and 3/5 on anti-IL1 treatment. Another 8 patients were diagnosed with either FMF (n=6) or PFAPA syndrome (n=2) presenting with prominent diarrhea (n=7), oral ulcers (n=2), periorbital swelling and sicca-like symptoms (n=1), or maculopapular rash (n=1). One patient had a clinically undefined SAID, albeit characterized by oral ulcers and diarrhea. Finally, one patient presented with chronic relapsing urticaria with periorbital edema and inflammatory markers, and another one had a Crohn-like syndrome with good response to anti-IL-1 but refractory to anti-TNF treatment. Conclusion: NOD2 variants were detected in 1 out of 7 SAID patients and seem to have an impact on disease phenotype and treatment response. Further studies should validate combined molecular and clinical data to better understand these distinct nosological entities.


Assuntos
Exantema , Doenças Hereditárias Autoinflamatórias , Úlceras Orais , Síndrome de Imunodeficiência Adquirida dos Símios , Adulto , Animais , Humanos , Doenças Hereditárias Autoinflamatórias/diagnóstico , Doenças Hereditárias Autoinflamatórias/tratamento farmacológico , Doenças Hereditárias Autoinflamatórias/genética , Estudos Retrospectivos , Inibidores do Fator de Necrose Tumoral/uso terapêutico , Diarreia/etiologia , Proteína Adaptadora de Sinalização NOD2/genética
3.
Curr Rheumatol Rev ; 20(4): 451-454, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38243962

RESUMO

BACKGROUND: Tumor necrosis factor alpha (TNFα) is a pivotal cytokine involved in the pathogenesis of certain inflammatory diseases, such as rheumatoid arthritis (RA), spondyloarthropathies, and inflammatory bowel diseases. In the last two decades, TNFα inhibitors (TNFi) have revolutionized the treatment and outcome of the above disorders. However, the use of TNFi has been associated with the development of many autoimmune phenomena and paradoxical skin manifestations that may present as the same type of clinical indications for which the TNFi effectively used. Thus, they may display as arthritis, uveitis, colitis, psoriasis, and several other cutaneous clinical manifestations, among them the development of morphea, a localized scleroderma skin lesion. CASE PRESENTATION: We describe a 58-year-old woman with seronegative RA, refractory to methotrexate, who was treated with ABP-501 (Hefiya), an adalimumab (ADA) biosimilar and developed an oval-shaped, deep skin lesion of approximately 3.5cm in size, affecting the left part of her back compatible with morphea 3 months after the initiation of therapy. ADA biosimilar was discontinued and two months later, she had substantial skin improvement. CONCLUSION: This is the first report of morphea manifestation during TNFi biosimilar since the patient had no other trigger factors for morphea development like trauma and infections. Physicians dealing with patients treated with TNFi biosimilars should be aware of paradoxical skin reactions, among them morphea; thus, close monitoring, a minute and careful clinical examination, and a follow- up check are required.


Assuntos
Adalimumab , Antirreumáticos , Medicamentos Biossimilares , Esclerodermia Localizada , Humanos , Feminino , Esclerodermia Localizada/induzido quimicamente , Esclerodermia Localizada/tratamento farmacológico , Pessoa de Meia-Idade , Adalimumab/efeitos adversos , Adalimumab/uso terapêutico , Medicamentos Biossimilares/uso terapêutico , Medicamentos Biossimilares/efeitos adversos , Antirreumáticos/efeitos adversos , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/complicações
4.
Rheumatol Int ; 2024 Jan 31.
Artigo em Inglês | MEDLINE | ID: mdl-38294543

RESUMO

OBJECTIVE: This study aims to evaluate the active and chronic lesions in sacroiliac joints and lumbar spine over a decade of TNFi therapy in patients with AS. METHODS: The study enrolled patients with AS under treatment with a TNFi for over a decade. The patients underwent a new MRI scan of their lumbar spine and sacroiliac joint (SIJ). Two readers evaluated all images. Inflammation of SIJ (SIS), SIJ structural damage (SSS) including Fat Metaplasia, Erosions, Backfill and Ankylosis, and Spondyloarthritis Research Consortium of Canada Bone marrow edema (SPARCC) spine score were recorded. RESULTS: In the study, 15 patients were included, with 80% being male. The mean age during their first MRI was 38.1 (± 11.9) years old, and the majority (86.7%) tested positive for HLA-B27. While TNFi improved both BASDAI and BASFI scores, there was a noticeable increase in MRI acute lesions in the SIJ over time, where the median score increased from 0 (0-4) to 3 (0-10) after ten years (p = 0.028). After a decade of treatment, the median SPARCC spine score also increased from 0 (0-9) to 5 (0-16), p = 0.093. Finally, it was observed that there was a significant positive correlation between ESR and SIS erosions in cases of chronic lesions (r = 0.819, p < 0.001). CONCLUSIONS: While TNFi have significantly improved the treatment of AS, this study shows that acute lesions can still develop despite treatment. A personalized approach that adapts MRI assessment to each patient's specific requirements may help detect changes early and enable doctors to intervene promptly to prevent further damage.

5.
Semin Arthritis Rheum ; 63: 152272, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37788595

RESUMO

In recent years, identifying the pathophysiologic mechanisms underlying autoimmune arthritides and systematic diseases has led to the use of biological drugs. The primary targets of those biological therapies are cytokines, B cells, and co-stimulation molecules. So far, these targeted therapies have shown good clinical improvement and an acceptable toxicity profile. However, by blocking components of an intact immune system, autoimmune phenomena and paradoxical inflammation have emerged, and among them many cutaneous immune-related adverse events (irAEs). In this article, we review the current state of knowledge on the clinical features and mechanisms of specific cutaneous irAEs observed during treatment with biological therapies. Among those, psoriatic skin lesions are the most commonly observed. Herein, we also report new cases of cutaneous irAEs recently seen in our clinic to help physicians treating inflammatory arthritides recognize cutaneous irAEs early and better manage patients receiving biologic therapies.


Assuntos
Artrite , Neoplasias , Humanos , Terapia Biológica/efeitos adversos , Pele , Citocinas , Neoplasias/tratamento farmacológico
6.
Eur J Intern Med ; 117: 21-27, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37414646

RESUMO

Spondyloarthritis (SpA) is a chronic inflammatory disease that affects the axial skeleton (axSpA) and/or the peripheral joints (p-SpA) and entheses. The natural history of SpA in the decades of the 80 and 90 s involved a progressive disease with pain, spinal stiffness, ankylosis of the axial skeleton, structural damage of peripheral joints, and a poor prognosis. In the last 20 years, enormous advances in understanding and managing SpA have occurred. With the introduction of the ASAS classification criteria and MRI, early disease recognition is now possible. The ASAS criteria widened the spectrum of SpA to include all the disease phenotypes, such as radiographic (r-axSpA), non-radiographic (nr-axSpA), and p-SpA and extraskeletal manifestations. Nowadays, the treatment of SpA is based on a shared decision between patients and rheumatologists and includes non-pharmacological and pharmacological therapies. Moreover, the discovery of TNFα, IL-17, which play a pivotal role in disease pathophysiology, has revolutionized disease management. Thus, new targeted therapies and many biological agents are now available and used in SpA patients. TNFα inhibitors (TNFi), IL-17, and JAK inhibitors were proven to be efficacious, with an acceptable toxicity profile. Overall, their efficacy and safety are comparable with some differences. Sustained clinical disease remission, low disease activity, improvement of patient's quality of life, and prevention of progression of structural damage, are the results of the above interventions. The concept of SpA has changed in the last 20 years. The disease burden can be ameliorated by early and accurate diagnosis and targeting therapies.


Assuntos
Espondiloartrite Axial , Espondilartrite , Espondilite Anquilosante , Humanos , Espondilite Anquilosante/tratamento farmacológico , Fator de Necrose Tumoral alfa , Interleucina-17/uso terapêutico , Qualidade de Vida , Espondilartrite/diagnóstico , Espondilartrite/tratamento farmacológico
7.
Rheumatol Int ; 43(9): 1751-1754, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37316633

RESUMO

Relapsing polychondritis (RP) is a rare autoimmune disease characterized by inflammation of the cartilage structures of the body with typical features of auricular chondritis, nasal and ocular inflammation, audio-vestibular damage, as well as respiratory tract manifestations. It is associated with several autoimmune diseases and many other disorders. Tumor necrosis factor alpha (TNFα) inhibitors treat many chronic inflammatory disorders. They have proven effective and relatively safe in many clinical trials and observational studies. However, several autoimmune phenomena and paradoxical inflammation have been described with TNFα inhibitors, among them RP. This report presents a 43-year-old man with psoriatic arthritis treated with ABP-501 (Amgevita), an adalimumab (ADA) biosimilar and who developed RP, 8 months after the initiation of the treatment. This, is the first report of RP development during TNFα inhibitors biosimilar. We concluded that rheumatologists dealing with patients treated with TNFα inhibitors (originators or biosimilars), should be aware of several paradoxical reactions which may emerge and RP, is one of them.


Assuntos
Doenças Autoimunes , Medicamentos Biossimilares , Policondrite Recidivante , Masculino , Humanos , Adulto , Medicamentos Biossimilares/efeitos adversos , Fator de Necrose Tumoral alfa/uso terapêutico , Doenças Autoimunes/complicações , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Inflamação/complicações
8.
Expert Rev Clin Immunol ; 18(9): 923-931, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35904251

RESUMO

INTRODUCTION: Rheumatoid arthritis (RA) is a chronic systemic disease characterized by articular involvement and extra-articular manifestations. The incidence and prevalence of the disease vary across populations, and there is an ongoing debate on whether a change of RA occurrence over time exists or is due to methodological issues and other biases. Moreover, the disease's onset is related to an interaction of genetic and environmental factors that influence its expression. AREAS COVERED: This review explores the latest knowledge on RA epidemiology and the possible risk factors associated with its presentation to identify potential warning signs that may in the future help disease management. EXPERT OPINION: Current epidemiological evidence suggests a significant impact of smoking, sex hormones, and lifestyle status in RA occurrence. However, the association between these variables has not yet been thoroughly studied. Still, their effect must be interpreted as they may present subsequently integral indicators for a more rational approach of the disease.


Assuntos
Artrite Reumatoide , Artrite Reumatoide/complicações , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/genética , Humanos , Incidência , Prevalência , Fatores de Risco , Fumar/epidemiologia
9.
Expert Rev Clin Immunol ; 18(5): 485-493, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35535405

RESUMO

INTRODUCTION: In recent decades, Rheumatoid arthritis (RA) treatment landscape has evolved with the induction of new biological and targeted therapies that provide significant therapeutic benefits in patients with sustained disease. AREAS COVERED: Tumor necrosis factor inhibitors (TNFi) were the first biologics used in the treatment of RA. Although they present a significant efficacy, an insufficient response of some patients led to further research and discovery of targeted therapies, such as Janus kinase inhibitors (JAKi), which act at a molecular level, regulating many cytokines. Clinical benefits have been seen with both TNFi and JAKi as monotherapy and combined with conventional synthetic disease-modifying antirheumatic drugs. Still, some significant side effects have been reported with JAKi, and several questions remain about their safety and selectivity in action. This review summarizes the current knowledge on the mechanism of action, the clinical efficacy, and safety of TNFi vs. JAKi. EXPERT OPINION: TNFi and JAKi are particularly useful in treating inflammatory arthropathies. Both drug categories are recommended by ACR and EULAR institutions in RA patients suffering from moderate to severe disease. Safety data in long-term studies are required to determine the optimal benefit to the risk profile of JAKi use.


Assuntos
Antirreumáticos , Artrite Reumatoide , Inibidores de Janus Quinases , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Humanos , Inibidores de Janus Quinases/uso terapêutico , Janus Quinases , Inibidores do Fator de Necrose Tumoral
10.
PLoS One ; 17(3): e0264388, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35245310

RESUMO

BACKGROUND: Nationwide data on thyroid disease prevalence in Greece is lacking. Using the national health examination survey EMENO data resources, we aimed to estimate the prevalence of hypothyroidism and hyperthyroidism and associated risk factors in adults living in Greece. METHODS: A random sample of the adults (≥18 years) living in Greece was drawn by multi-stage stratified random sampling based on the 2011 census. During home visits, trained interviewers administered a standardized questionnaire to study participants. All participants answered questions concerning demographic parameters (e.g., age, sex, degree of urbanization, income) and questions concerning smoking habits, alcohol, dietary habits and psychological parameters such as anxiety and thyroid disease. Weighted logistic regression models were fitted to assess factors associated with thyroid disease. RESULTS: In total, 6006 individuals were recruited in the Greek Health Examination Survey EMENO (response rate 72%) of whom 5981 were eligible for this study. The prevalence of thyroid disease was 9%, where 0.4% was related to hyperthyroidism and 8.6% to hypothyroidism. The prevalence of thyroid disease was higher in women (14.9%) than men (2.7%) (p<0.001). The highest rates of thyroid disease were observed in former iodine-deficient areas. A decrease in the prevalence of thyroidopathies with increasing alcohol consumption was found. Thyroid disease was associated with anxiety in men. Multivariable regression analysis showed that age, geographic area, and smoking were related to thyroid disease. CONCLUSION: The prevalence of thyroid disease in Greece is higher in women. Age, habits, and characteristics of geographic areas determine the distribution of thyroidopathies in Greece.


Assuntos
Hipertireoidismo , Hipotireoidismo , Doenças da Glândula Tireoide , Adulto , Estudos Transversais , Feminino , Grécia/epidemiologia , Humanos , Hipertireoidismo/epidemiologia , Hipotireoidismo/epidemiologia , Masculino , Prevalência , Doenças da Glândula Tireoide/epidemiologia
11.
Clin Exp Rheumatol ; 40(9): 1642-1649, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34796838

RESUMO

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.


Assuntos
Fibrose Retroperitoneal , Proteínas de Fase Aguda , Doença Crônica , Feminino , Grécia , Humanos , Imunoglobulina G , Masculino , Recidiva , Fibrose Retroperitoneal/diagnóstico por imagem , Fibrose Retroperitoneal/tratamento farmacológico , Estudos Retrospectivos
12.
Clin Rheumatol ; 40(11): 4741-4748, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33782750

RESUMO

Psoriatic arthritis (PsA) is a chronic inflammatory disease associated with psoriasis. Pulmonary involvement is a rare extra-articular manifestation of the disease characterized by rigidity of the chest wall and apical pulmonary fibrosis. Pleural effusion is uncommon in PsA. We present four cases of patients with PsA who developed pleural effusions. We report for the first time a PsA patient who was drug-naïve and developed unilateral pleuritis. We also describe one PsA case with pleuritis while he was on methotrexate (MTX) and two PsA cases on tumor necrosis factor (TNF) inhibitors. The literature review revealed six cases with pleural effusion, which were drug-induced. These patients presented pleural effusions while they were treated with MTX (2 patients) and TNF inhibitors (4 patients). In PsA patients with pleuritis, a detailed investigation to rule out infections is necessary. In addition, increased pharmacovigilance will detect cases of drug-induced serositis.


Assuntos
Artrite Psoriásica , Derrame Pleural , Pleurisia , Artrite Psoriásica/complicações , Artrite Psoriásica/tratamento farmacológico , Humanos , Masculino , Metotrexato/efeitos adversos , Pleurisia/complicações , Inibidores do Fator de Necrose Tumoral/efeitos adversos
13.
Mediterr J Rheumatol ; 31(2): 235-236, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32676565

RESUMO

Psoriatic arthritis (PsA) is an inflammatory form of arthritis that belongs to the family of spondyloarthritis (SpA) and is related to skin psoriasis. The incidence and prevalence of the disease vary considerably between countries. PsA is classified into axial PsA and peripheral PsA, with a wide range of other extra-articular manifestations. Although the aetiology of the disease is unknown, genetic, environmental, and immunologic factors appear to affect its appearance. In recent years, the role of the immune system in the pathogenesis of PsA has been increasingly investigated. Specific cytokines such as tumour necrosis factor (TNF), interleukin (IL-) 17 and IL-23, play an essential role affecting joint structures. This observation led to the emergence of tumour necrosis factor inhibitors (TNFi) that offer considerable therapeutic benefit to PsA patients. However, chronic inflammation causes bone loss, while new bone formation may also occur in both peripheral and axial skeleton. The molecular mechanisms underlying these processes have not yet been fully understood. So far, the role of the Wnt/ß-catenin pathway and its inhibitors (Dickkopf and sclerostin) has been evaluated in ankylosing spondylitis (AS), but in PsA has not been studied sufficiently. The present study aims to investigate the epidemiological characteristics and clinical features (articular and extra-articular manifestations) as well as the treatment of PsA patients in the region of northwestern (NW) Greece. It also aims to evaluate the role of specific cytokines and sclerostin in patients with PsA, giving evidence to possible future biomarkers or even therapeutic targets for the disease.

14.
Rheumatol Int ; 40(8): 1181-1191, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32524301

RESUMO

Rheumatoid arthritis (RA) is an autoimmune disease characterized by chronic inflammation that, if left untreated, can cause joint destruction and physical impairments. The inflammatory process is systematic, and it is associated with increased morbidity and mortality. Over the last years, mortality presents a decreasing trend; still, there is a high burden of cardiovascular disease (CVD) in RA that seems to be related to coronary atherosclerosis. Chronic inflammation, physical inactivity, and drugs used to treat RA are some of the reasons. Thus, the management of CVD risk is essential and involves the patient's stratification using distinct parameters that include assessment of the blood lipid profile. However, 'dyslipidemia' in RA patients follows a different pattern under the impact of inflammatory processes, while therapies that target the underlying disease change the levels of specific lipid components. In this review, we explore the relationship between blood lipids and inflammation in the so-called ΄lipid paradox΄ in RA, and we present the existing knowledge over the influence of antirheumatic drugs on the lipid profile of RA patients.


Assuntos
Artrite Reumatoide/complicações , Doenças Cardiovasculares/etiologia , Dislipidemias/etiologia , Corticosteroides/administração & dosagem , Corticosteroides/efeitos adversos , Antirreumáticos/administração & dosagem , Antirreumáticos/efeitos adversos , Artrite Reumatoide/sangue , Artrite Reumatoide/tratamento farmacológico , Progressão da Doença , Dislipidemias/sangue , Dislipidemias/metabolismo , Fatores de Risco de Doenças Cardíacas , Humanos , Metabolismo dos Lipídeos , Inibidores do Fator de Necrose Tumoral/administração & dosagem , Inibidores do Fator de Necrose Tumoral/efeitos adversos
15.
Clin Exp Rheumatol ; 37 Suppl 118(3): 175-184, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31464663

RESUMO

Primary Sjögren's syndrome (pSS) is a chronic, systemic autoimmune disease with diverse clinical picture and outcome. The disease affects primarily middle-aged females and involves the exocrine glands leading to dry mouth and eyes. When the disease extends beyond the exocrine glands (systemic form), certain extraglandular manifestations involving liver, kidney, lungs, peripheral nervous system and the skin may occur. Primary SS is considered the crossroad between autoimmunity and lymphoproliferation, since approximately 5% of patients develop NHL associated lymphomas. As with every chronic disease with complex aetiopathogenesis and clinical heterogeneity, pSS has certain unmet needs that have to be addressed: a) classification and stratification of patients; b) understanding the distinct pathogenetic mechanisms and clinical phenotypes; c) defining and interpreting the real needs of patients regarding the contemporary diagnostic and therapeutic approaches; d) physician and patients' training regarding the wide spectrum of the disease; e) creating common policies across European countries to evaluate and manage SS patients. To achieve these goals, an intense effort is being currently undertaken by the HarmonicSS consortium in order to harmonise and integrate the largest European cohorts of pSS patients. In this review, we present an overview of our perception and vision, as well as new issues arising from this project such as harmonisation protocols and procedures, data sharing principles and various ethical and legal issues originating from these approaches.


Assuntos
Medicina de Precisão/métodos , Síndrome de Sjogren , Xerostomia , Autoimunidade , Europa (Continente) , Feminino , Humanos , Pessoa de Meia-Idade , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/genética
16.
Autoimmun Rev ; 17(11): 1124-1133, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30213691

RESUMO

OBJECTIVES: This study aimed to investigate whether positive serum autoantibodies (AAbs) have any impact on survival and time evolution of radiological findings and pulmonary function indices in patients with interstitial lung disease (ILD). PATIENTS AND METHODS: Ninety four patients with regular clinical, functional and high resolution computed tomography (HRCT) imaging follow-up for at least 12 consecutive months and complete testing for a panel of AAbs most commonly associated with ILD were enrolled in this retrospective two-center study. Eligible patients were divided into two groups based on the presence [ILD/AAb(+)] (n = 69) or absence [ILD/AAb(-)] (n = 25) of positive serum AAbs. All-cause mortality and longitudinal indicators of ILD progression such as a sustained decrease from baseline in absolute measurements of forced vital capacity (FVC) of ≥10% or single-breath diffusion capacity (DLCOSB) of ≥15% were the primary study endpoints. DLCOSB < 40% predicted on at least two consecutive measurements and progression of HRCT findings were our secondary endpoints. Kaplan-Meier (K-M) survival analysis and multivariate Cox proportional-hazards (PH) model were used to evaluate the prognostic significance of positive AAbs in the outcome of patients with ILD. RESULTS: ILD/AAb(+) patients were predominantly female (71% vs 32%), were significantly younger (54.8 ±â€¯14.6 vs 66.8 ±â€¯10.1 years), and had longer duration of follow-up (78.1 ±â€¯53.1 vs 41.6 ±â€¯26.7 months), compared with ILD/AAb(-) patients (p < .01 for each comparison). Baseline measurements of FVC (% pred.) and DLCOSB (% pred.) did not differ significantly between the two groups. At the end of follow-up, mortality rates and the percentage of patients with a sustained FVC decrease were lower in the ILD/AAb(+) group (p < .05 for each comparison). With the exception of DLCOSB < 40% pred., ILD/AAb(+) patients had a longer median time-to-event for each of the other studied outcomes (p < .01 for each K-M analysis). In addition, Cox PH models adjusted for age, smoking status, baseline pulmonary function tests and morphological pattern of ILD remained statistically significant in favor of the ILD/AAb(+) group (p < .05 for each comparison). CONCLUSIONS: AAb(+) patients with ILD seem to have a more favorable prognosis regarding all-cause mortality, long-term deterioration in lung function parameters and progression of HRCT findings than their AAb (-) counterparts.


Assuntos
Autoanticorpos/sangue , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/mortalidade , Pulmão/fisiopatologia , Idoso , Autoanticorpos/imunologia , Progressão da Doença , Feminino , Humanos , Doenças Pulmonares Intersticiais/imunologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Taxa de Sobrevida , Capacidade Vital
17.
Semin Arthritis Rheum ; 37(5): 293-8, 2008 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17888499

RESUMO

OBJECTIVE: To investigate the efficacy, toxicity, and survival of infliximab in patients with psoriatic arthritis (PsA). METHODS: Thirty-two patients with PsA, refractory to at least 2 disease-modifying antirheumatic drugs, were included in this prospective, open-label, uncontrolled study. All had active disease, defined as having a tender or swollen joint count > or =6, Psoriasis Area and Severity Index (PASI) scores > or =10, and erythrocyte sedimentation rate > or =28 mm Hg/h, or C-reactive protein > or =10 mg/L. The primary endpoints were the percentage of patients who achieved the Psoriatic Arthritis Response criteria (PsARC) and the improvement of PASI. Patients were treated with infliximab (5 mg/kg) at weeks 0, 2, 6, and every 8 weeks thereafter for a period of 3 years. Data concerning infliximab efficacy, tolerability, concomitant therapy, adverse events, and drug discontinuation were recorded. The clinical response according to the American College of Rheumatology (ACR) criteria as well as the disease activity for 28 joint indices score (DAS-28) were also recorded. RESULTS: After the third year of treatment, PsARC was achieved by 23/32 of patients, PASI 70 by 24/32, and PASI 90 by 23/32. A significant improvement of ACR and DAS-28 was noted. Clinical improvement was associated with a reduction of acute phase reactants. Eight patients withdrew from the study primarily for acute allergic reactions. After the first year, infliximab survival was 84%, while after the second year, it was 75%, which was maintained throughout the third year of treatment. CONCLUSION: Infliximab was effective, safe, and well tolerated in patients with PsA. The clinical response was maintained for a period of 3 years with high infliximab survival.


Assuntos
Anti-Inflamatórios/administração & dosagem , Anticorpos Monoclonais/administração & dosagem , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/mortalidade , Preparações de Ação Retardada , Relação Dose-Resposta a Droga , Feminino , Seguimentos , Grécia/epidemiologia , Humanos , Incidência , Infliximab , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do Tratamento , Fator de Necrose Tumoral alfa
18.
Rheumatol Int ; 27(10): 935-9, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17357804

RESUMO

Our aim was to investigate the efficacy, toxicity, and drug discontinuation in patients with ankylosing spondylitis (AS) treated with infliximab. Thirty-five patients with AS, who were enrolled between June 2001 and December 2002 were treated with infliximab. All patients fulfilled the New York revised criteria for AS and had axial disease. Infliximab (5 mg/kg weight), was given intravenously at weeks 0, 2, 6, and every 8 weeks thereafter. If this failed to give an acceptable treatment response, the interval was shortened to 6 or 4 weeks. The patients were followed-up at predefined times according to a standardized protocol. Data concerning infliximab efficacy, tolerability, adverse events, interval, and drug discontinuation were all recorded. Clinical improvement according to the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) 50% and the Ankylosing Spondylitis Assessment Study group (ASAS) 40%, and ASAS 5/6 response criteria were recorded. Infliximab treatment resulted in a rapid improvement in the BASDAI and ASAS scores in the first year of the treatment, which sustained throughout the fourth year. More specifically, after the third year of treatment 17/35 (48.6%) of patients achieved BASDAI 50% response criteria, 19/35 (54.3%) attained the ASAS 40% and 15/35 (42.9%) reached the ASAS 5/6. After the fourth year of treatment BASDAI 50% was reached by 17/35 (48.6%) of patients, ASAS 40% by 17/35 (48.6%), while ASAS 5/6 was attained by 15/35 (42.9%). The clinical improvement was associated with the reduction of acute phase reactants as measured by C-reactive protein levels. After the first year of treatment, the "survival rate" of infliximab was 94.3%, after the second year was 91.4%, after the third year was 85.7% and even after 4 years of treatment still maintained high 77.9%. Six (17.1%) patients were withdrawn during the observational period. Three because of lack of efficacy, two because of allergic reactions and one lost from follow-up. Infliximab was effective, safe, and well tolerated in patients with AS. The clinical response was maintained for a period of 4 years and over, with infliximab survival of 77.9%.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Fatores Imunológicos/uso terapêutico , Espondilite Anquilosante/tratamento farmacológico , Adulto , Anticorpos Monoclonais/efeitos adversos , Feminino , Humanos , Fatores Imunológicos/efeitos adversos , Infliximab , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Cooperação do Paciente , Índice de Gravidade de Doença , Resultado do Tratamento , Fator de Necrose Tumoral alfa/antagonistas & inibidores
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