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3.
Int J Rheum Dis ; 23(2): 256-261, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31976619

RESUMO

OBJECTIVES: Vascular involvement is one of the major causes of morbidity and mortality in Behçet's disease (BD) patients. Immunosuppressive (IS) agents are the mainstay of vascular BD (VBD) treatment; however, up to one-third of patients relapse under conventional ISs. In this case series, we present the results of tumor necrosis factor-alpha (TNFα) inhibitor use for the treatment of VBD patients who were refractory to conventional ISs and corticosteroids. METHODS: This retrospective multicenter study included 27 refractory VBD patients treated with TNFα inhibitor agents. All data were acquired from patient charts. RESULTS: Complete clinical remission was achieved in 22 (80%) patients within 3 months of the initiation of TNFα inhibitors. Infliximab was the first choice of TNFα inhibitor in 24 and adalimumab in three patients. The median daily dose of corticosteroids significantly decreased at 3 months. A trend toward a higher rate of complete remission was observed with concomitant IS use compared to monotherapy of TNFα inhibitors (93% vs 67%, P = .09). Serious side effects were observed in two patients (one pneumonia and one tuberculosis). CONCLUSION: Tumor necrosis factor-alpha inhibitors seem a highly effective option for remission-induction of refractory VBD with an acceptable safety data. Concomitant IS use may achieve higher complete remission rates as compared to TNFα inhibitor monotherapy. Comparative efficacy and safety of biological agents for VBD require further prospective, randomized controlled studies with a longer duration of follow-up.

4.
Ocul Immunol Inflamm ; 28(1): 86-91, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-30556792

RESUMO

Purpose: To describe posterior segment findings of antiphospholipid syndrome (APS) and compare them with systemic lupus erythematosus (SLE).Methods: A total of 11 patients with primary APS, 29 secondary APS patients, and 29 SLE patients without APS were included. All patients were referred from rheumatology clinic for detailed ophthalmologic examination. When patients had suspicious lesions, fundus fluorescein angiography was performed (n = 56).Results: The most common retinal examination finding was peripheral venous tortuosity (17.5%) in APS, which was not observed in SLE group. Common FFA findings were pigment epithelial window defects (10%) and vascular filling delays (7.5%) in APS, which were observed in 27.5% and 3.5% of patients with SLE consecutively.Conclusion: Venous tortuosity was significantly more in patients with APS. There was no significant difference for other ocular findings between the groups. Ocular complication rate was lower compared to earlier reports, probably due to better management of disease activity with current treatment protocols.

5.
Int J Rheum Dis ; 23(2): 240-246, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31858715

RESUMO

OBJECTIVE: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). METHODS: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. RESULTS: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration <5 years (74%) were higher in male patients with work-day loss (P < .05). The majority of males (90.9%) had more than four clinic visits during the previous year. Moreover, the mean work-day loss (30.8 ± 57.7 days) was higher in patients with vascular involvement (56.1 ± 85.9) than those without (26.4 ± 50.6 days) (P = .046). In addition, increased frequency of ocular involvement (25.9%) was also observed in patients with work-day loss compared to others (8.6%) (P = .059). CONCLUSION: Work-day loss was associated with both vascular and ocular involvement. Close associations were observed among male gender, early period of the disease, frequent medical visits, being a smoker and treatment with IS medications in patients with work-day loss.

6.
Clin Exp Rheumatol ; 37 Suppl 121(6): 98-104, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31856935

RESUMO

OBJECTIVES: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS). METHODS: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity. RESULTS: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05). CONCLUSIONS: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.


Assuntos
Síndrome de Behçet , Imunossupressores/uso terapêutico , Úlceras Orais , Síndrome de Behçet/classificação , Estudos Transversais , Progressão da Doença , Feminino , Humanos , Masculino , Úlceras Orais/classificação , Recidiva , Índice de Gravidade de Doença
7.
Clin Rheumatol ; 38(11): 3033-3039, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31270696

RESUMO

OBJECTIVE: To determine predictors and optimal duration of sustained remission (SR) in patients with rheumatoid arthritis (RA). METHODS: A total of 428 consecutive patients with RA visiting our clinic routinely between 2012 and 2013 were evaluated. Seventy seven of these patients in DAS28 remission were enrolled and followed up for 62.2 ± 9.9 months. Patients in remission ≥ 6 months (SR) and shorter (non: N-SR) were compared in terms of demographic-clinical data and the psychosocial factors. At enrollment, 1st and 5th years, patients in DAS28, SDAI, and Boolean remission were determined. RESULTS: Sixty three patients were in SR and 14 in N-SR. Lower baseline DAS28 and HAQ scores, anti-CCP were positive predictors of SR. Although the presence of anxiety, depression, fibromyalgia, and fatigue were lower in the SR group, there was no significance. Patients in DAS28 remission (100%) at baseline reduced to 64% at 1st and 42.6% at 5th years. Patients satisfying SDAI and Boolean remission at these three visits were 49%, 44%, and 32.4% vs 41%, 28%, and 20.6%, respectively. If the duration of remission is defined as 6 months, the remission rates of SDAI at inclusion and fifth years' visits were similar but Boolean remission rates differed significantly and if it is accepted as ≥ 12 months, both the SDAI and Boolean remission rates were not different. CONCLUSION: Low DAS28 and HAQ scores at baseline, anti-CCP were positive predictors of SR. Instead of 6 months, remission duration for ≥ 12 months would probably help us to predict SR independently from the chosen criteria; Boolean or SDAI.

8.
Clin Exp Rheumatol ; 37 Suppl 118(3): 140-145, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31287407

RESUMO

OBJECTIVES: To evaluate associations between salivary gland ultrasonography (SGUS) and clinical characteristics, disease activity and outcome in patients with primary Sjögren's syndrome (pSS). METHODS: The parotid and submandibular salivary glands were examined by ultrasonography using two different scoring systems proposed by Hocevar et al. and Milic et al. on 85 pSS patients. Patients with inhomogeneity/hypoechoic areas with scores ≥2 in parotid and submandibular glands were classified as severe parotid or severe submandibular involvements, respectively. Disease activity and patient-reported severity were evaluated using the European League Against Rheumatism Sjögren's Disease Activity Index (ESSDAI) and the European League Against Rheumatism Sjögren's Patient Reported Index (ESSPRI). Salivary gland functional capacity was investigated by unstimulated whole saliva flow rate (U-WSFR). RESULTS: Of the activity scores, ESSPRI dryness component was higher in pSS patients who had scores above the cut-off values for Hocevar (6.1±2.3 vs. 4.9±2.6, p=0.026). The patients with any type of systemic involvement more frequently showed higher SGUS scores, according to both Hocevar (72.4 vs. 44.6%, p=0.013) and Milic (75.9 vs. 51.8%, p=0.026). These patients also showed a higher percentage of severe parotid/submandibular changes on US imaging (65.5 vs. 33.9%, p=0.005 and 75.9 vs. 51.8%, p=0.026 respectively). Higher SGUS scores according to cut-off values of both scoring systems and severe parotid/submandibular involvements were associated with both anti-Ro or double anti-Ro/La autoantibodies and inversely associated with U-WSFR. CONCLUSIONS: SGUS may be a useful imaging modality for the selection of patients with more severe disease status or who may require a tight follow-up schedule.


Assuntos
Glândula Parótida/diagnóstico por imagem , Glândulas Salivares/diagnóstico por imagem , Síndrome de Sjogren , Glândula Submandibular/diagnóstico por imagem , Ultrassonografia/métodos , Humanos , Índice de Gravidade de Doença , Síndrome de Sjogren/diagnóstico por imagem
9.
Indian J Dermatol ; 64(3): 201-206, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31148858

RESUMO

Context: Advanced glycation end products (AGEs) promote oxidative stress and inflammation by altering structure and function of proteins. They are excessively produced mainly in hyperglycemia, chronic inflammation and are involved in the development of atherosclerosis and cardiovascular disease. Aims: The aim of this study was to investigate whether skin AGEs levels were increased and had relation to premature atherosclerosis in patients with psoriasis. Subjects and Methods: Fifty-two psoriasis patients and 20 healthy controls (HC) were included. AGEs were determined by skin autofluorescence (SAF) analysis. High-sensitive C-reactive protein (hsCRP) and carotid intima-media thickness (CIMT) were also investigated. Physical activity and dietary patterns were determined. Statistical Analysis Used: Fisher's exact test, two-sample t-tests, Mann-Whitney-U test, Pearson correlation, Spearman correlation, and Wilcoxon test. Results: SAFs were increased in psoriasis patients (1.8 arbitrary units [AUs]) compared to that in HC (1.6 AUs) (P = 0.057). Median CIMT values of HC and psoriasis groups were 0.43 (0.28-0.79), and 0.59 (0.44-0.98) respectively and the differences were significant (P = 0.001); hsCRP levels were not different between groups. Conclusions: Skin AGE accumulation was found to have a correlation with CIMT in psoriasis patients providing evidence for the role of AGEs in premature atherosclerosis.

10.
BMC Med Genomics ; 12(1): 58, 2019 05 02.
Artigo em Inglês | MEDLINE | ID: mdl-31046767

RESUMO

BACKGROUND: Human Endogenous Retroviruses type K HML-2 (HK2) are integrated into 117 or more areas of human chromosomal arms while two newly discovered HK2 proviruses, K111 and K222, spread extensively in pericentromeric regions, are the first retroviruses discovered in these areas of our genome. METHODS: We use PCR and sequencing analysis to characterize pericentromeric K111 proviruses in DNA from individuals of diverse ethnicities and patients with different diseases. RESULTS: We found that the 5' LTR-gag region of K111 proviruses is missing in certain individuals, creating pericentromeric instability. K111 deletion (-/- K111) is seen in about 15% of Caucasian, Asian, and Middle Eastern populations; it is missing in 2.36% of African individuals, suggesting that the -/- K111 genotype originated out of Africa. As we identified the -/-K111 genotype in Cutaneous T-cell lymphoma (CTCL) cell lines, we studied whether the -/-K111 genotype is associated with CTCL. We found a significant increase in the frequency of detection of the -/-K111 genotype in Caucasian patients with severe CTCL and/or Sézary syndrome (n = 35, 37.14%), compared to healthy controls (n = 160, 15.6%) [p = 0.011]. The -/-K111 genotype was also found to vary in HIV-1 infection. Although Caucasian healthy individuals have a similar frequency of detection of the -/- K111 genotype, Caucasian HIV Long-Term Non-Progressors (LTNPs) and/or elite controllers, have significantly higher detection of the -/-K111 genotype (30.55%; n = 36) than patients who rapidly progress to AIDS (8.5%; n = 47) [p = 0.0097]. CONCLUSION: Our data indicate that pericentromeric instability is associated with more severe CTCL and/or Sézary syndrome in Caucasians, and appears to allow T-cells to survive lysis by HIV infection. These findings also provide new understanding of human evolution, as the -/-K111 genotype appears to have arisen out of Africa and is distributed unevenly throughout the world, possibly affecting the severity of HIV in different geographic areas.


Assuntos
Centrômero/virologia , Retrovirus Endógenos/genética , Retrovirus Endógenos/fisiologia , Variação Genética , Infecções por HIV/virologia , Linfoma Cutâneo de Células T/virologia , Síndrome de Sézary/virologia , Animais , Linhagem Celular , Genótipo , Humanos
12.
J Rheumatol ; 46(9): 1198-1201, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30877212

RESUMO

OBJECTIVE: The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group seeks to develop validated outcome measures for use in trials for large-vessel vasculitis (LVV). METHODS: An international Delphi exercise conducted among investigators identified items considered important to measure active disease. In parallel, qualitative research with patients was conducted, including interviews and focus groups. RESULTS: Next steps prioritized by the group for LVV include (1) defining disease states (remission, flare, and patient-acceptable symptom state) and (2) selection of patient-reported outcome tools. CONCLUSION: The ultimate goal is to develop an OMERACT-endorsed core set of outcome measures for use in clinical trials of LVV.

13.
Turk J Med Sci ; 49(2): 472-477, 2019 04 18.
Artigo em Inglês | MEDLINE | ID: mdl-30887796

RESUMO

Background/aim: The distribution of Mediterranean fever (MEFV) gene mutations in Turkish familial Mediterranean fever (FMF) patients varies according to geographic area of Turkey. There is a need for highly representative data for Turkish FMF patients. The aim of our study was to investigate the distribution of the common MEFV mutations in Turkish FMF patients in a nationwide, multicenter study. Materials and methods: Data of the 2246 FMF patients, from 15 adult rheumatology clinics located in different parts of the country, were evaluated retrospectively. The following mutations have been tested in all patients: M694V, M680I, M694I, V726A, and E148Q. Results: There were 1719 FMF patients with available genetic testing. According to the genotyping, homozygous M694V, present in 413 patients (24%), was the most common mutation . One hundred and fifty-four (9%) of patients had no detectable mutations. Allele frequencies of common mutations were: M694V (n = 1529, 44.5%), M680I (n = 423, 12.3%), V726A (n = 315, 9.2%), E148Q (n = 214, 1%), and M694I (n = 12, <1%). Conclusion: In this large-scale multicenter study, we provided information about the frequencies of common MEFV gene mutations obtained from adult Turkish FMF patients. Nearly half of the patients were carrying at least one M694V mutations in their alleles.


Assuntos
Proteínas do Citoesqueleto/genética , Febre Familiar do Mediterrâneo/genética , Genética Populacional , Mutação/genética , Pirina/genética , Adolescente , Adulto , Criança , Análise Mutacional de DNA , Febre Familiar do Mediterrâneo/diagnóstico , Feminino , Frequência do Gene , Predisposição Genética para Doença/epidemiologia , Testes Genéticos , Genética Populacional/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Taxa de Mutação , Estudos Retrospectivos , Turquia/epidemiologia , Adulto Jovem
14.
Clin Rheumatol ; 38(5): 1447-1451, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30758790

RESUMO

Vascular involvement, especially in young males, is seen in up to 40% of the patients with Behcet's disease (BD) and is a major cause of mortality and morbidity. In this study, we investigated vessel wall thickness (VWT) and dilatation in lower extremity veins with Doppler ultrasound (US) in male BD patients. Sixty-one male patients with BD, 37 healthy male controls (HC) and 27 male patients, with ankylosing spondylitis (AS), were included in the study. Venous Doppler US was performed by an experienced radiologist blinded to cases. Bilateral common femoral vein (CFV) wall thickness and great/small saphenous vein (SV) dilatations were assessed. All venous measurements were significantly higher in BD compared to AS and HC (p < 0.001 for all). Both right and left extremity CFV thicknesses had a high area under the ROC curve (> 0.8). Cut-off values for right and left CFV thicknesses for BD was 0.49 and 0.48 mm, respectively. High sensitivity and specificities are observed for both measurements (right CFV: sensitivity 81%, specificity 78.4%; left CFV: sensitivity 82.8%, specificity 81.1%). We found increased CFV thickness in BD patients independent of vascular involvement. As a similar change was not observed in controls, increased CFV thickness may be a specific sign of venous inflammation in BD. Our acceptable sensitivity and specificity values of CFV measurements suggest that assessment of femoral vein thickness with US may be a candidate diagnostic tool, especially in young males suspected of BD.


Assuntos
Síndrome de Behçet/diagnóstico por imagem , Veia Femoral/patologia , Espondilite Anquilosante/diagnóstico por imagem , Doenças Vasculares/diagnóstico por imagem , Adulto , Síndrome de Behçet/complicações , Estudos Transversais , Veia Femoral/diagnóstico por imagem , Humanos , Extremidade Inferior/diagnóstico por imagem , Masculino , Curva ROC , Índice de Gravidade de Doença , Espondilite Anquilosante/complicações , Turquia , Ultrassonografia Doppler , Doenças Vasculares/complicações , Trombose Venosa/diagnóstico
15.
Eur J Rheumatol ; 6(1): 34-37, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30489254

RESUMO

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. METHODS: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. RESULTS: Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. CONCLUSION: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

16.
Intern Emerg Med ; 14(5): 691-697, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30506212

RESUMO

This study aims to assess the relationship between illness perception and disease course and symptoms in Behçet disease (BD). One hundred ten consecutive BD patients (F/M 50/60, mean age 38.5 ± 9.88 years) and 57 patients with Psoriasis as a disease control group (F/M 28/29, mean age 48.12 ± 15.52) are included in this cross-sectional study. Illness perception is evaluated using a revised version of the Illness Perception Questionnaire (IPQ-R). In IPQ-R, the identity score reflecting the number of symptoms is higher in BD patients with musculoskeletal involvement than the others (6.77 ± 2.91 vs. 5.08 ± 3.3, respectively, p = 0.007). The consequences score for musculoskeletal involvement (19.52 ± 7.03) and timeline (acute/chronic) score for eye involvement (26.67 ± 4.32) are also higher compared to patients without them (16.37 ± 5.82 and 22.09 ± 8.68) (p = 0.011 and p = 0.038), reflecting negative beliefs about the illness. The score of psychological attribution is higher in patients with psoriasis than BD (p = 0.039), whereas the other subgroup scores are lower in patients with psoriasis compared to those of BD (p < 0.05). This study provides a patient's perspective in the disease management process of BD using the IPQ-R questionnaire. A patient's own personal beliefs and emotional responses to their symptoms might affect the outcome measures, especially with musculoskeletal symptoms and eye involvement in BD. However, psychological attribution is found to be a prominent issue in psoriasis.

17.
Intern Emerg Med ; 14(5): 653-660, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30523495

RESUMO

Environmental and other triggering factors are suggested to cause the onset and the clinical relapses of Behçet's syndrome (BS), a multi-systemic inflammatory disorder. In this review, environmental factors are discussed according to their interactions with etiopathogenesis, immune response and disease activity. Stress is a common self-triggering factor for most BS patients. Stimuli such as some foods can activate oral ulcers, and may be linked to the histamine content of the food. Oral/skin trauma and menstruation associated with hormonal factors aggravate, whereas allergy/atopy seem to alleviate the symptoms of BS. Infections are associated with BS, and microbial stimuli can activate inflammation in mucosal surfaces with increased Th1/Th17 responses. Fecal and oral microbiome patterns change in diversity and composition in BS. Better oral hygiene applications and anti-microbial interventions might be helpful to suppress oral ulcers in BS.

18.
J Oral Rehabil ; 46(4): 349-354, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30466164

RESUMO

OBJECTIVES: The aim of the study was to examine whether oral health as an infection focus could mediate disease course in patients with Behçet's disease (BD). METHODS: In the study, oral health of 194 BD patients was examined at baseline and follow-up periods. The reasons for last dental visits were recorded as tooth extraction or regular control visits/planned treatments at the end of follow-up period. The Behçet's disease severity score was calculated with higher scores indicating a more severe course. Mediation analysis was carried out to assess the effects of oral health on disease severity score at follow-up period in the study. RESULTS: Dental and periodontal indices were found to be higher at follow-up visit compared to those of baseline (P < 0.05). Disease severity score was found to be higher in males (5.3 ± 2.4) compared to females (4.4 ±2.5) in the whole group (P = 0.005). Moreover, patients having tooth extraction at their last dental visit and patients with dental caries had a more severe disease course (5.4 ± 2.4; 5.5 ± 2.5) compared to others (4.2 ± 2.3; 4.4 ± 2.4; P < 0.0001). In multiple mediation analysis, disease severity score was a dependent variable and was directly mediated by male gender (B = -0.8822, P = 0.0145) and indirectly mediated through the presence of dental caries (B = 0.9509 P = 0.0110) and need of tooth extraction (B = 0.8758, P = 0.0128). CONCLUSION: Both presence of dental caries and need of tooth extraction were observed to be effective mediators for a more severe disease course in BD. Therefore, better oral health should be aimed to eliminate microbial factors, which are a part of pathogenic processes.


Assuntos
Síndrome de Behçet/patologia , Síndrome de Behçet/fisiopatologia , Cárie Dentária/epidemiologia , Saúde Bucal/estatística & dados numéricos , Extração Dentária/estatística & dados numéricos , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/epidemiologia , Cárie Dentária/complicações , Índice de Placa Dentária , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Estatísticos , Índice Periodontal , Prevalência , Psicometria , Estudos Retrospectivos , Distribuição por Sexo , Escovação Dentária/normas , Turquia/epidemiologia
19.
J Ocul Pharmacol Ther ; 35(1): 58-65, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30359208

RESUMO

PURPOSE: To investigate factors affecting relapse and remission in patients with Behçet uveitis (BU) in the context of using the interferon alpha2a (IFNα2a) therapy. METHODS: Clinical records were reviewed of 32 patients (totaling 54 eyes) presenting with severe BU who had been treated with IFNα2a therapy. Factors influencing relapsing-remitting course of BU during IFNα2a therapy and after the cessation of the agent were evaluated. Possible affecting factors of recurrence were age, gender, duration of the Behçet disease, time elapsed before starting the drug, number of relapses before the starting of IFNα2a, laterality, and presence of panuveitis. RESULTS: Thirty patients (93.7%) responded to IFNα2a therapy. Annual attack rate before and after IFNα2a treatment was determined to be 4.03 ± 3.19 (attack per year) and 0.58 ± 1.07, respectively (P < 0.001). In univariable statistical evaluation, women tended to have a lower relapse risk than men. Multivariable analyses showed that age was the major factor determining relapse rate, while the effect of gender was not statistically significant (P = 0.196). The other probable influencing factors were not found to be statistically significant (P > 0.05). Increase in visual acuity observed during last examination compared to initial examination was determined to be statistically significant (P = 0.018). CONCLUSIONS: Even if the female gender was evaluated as a good prognostic factor in univariable analyses, it was not assessed as an effective prognostic factor in multivariable analyses. Being aged may be regarded a good prognostic factor on the relapsing-remitting course of patients with BU who were treated with IFNα2a therapy.

20.
Rheumatology (Oxford) ; 58(2): 289-298, 2019 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-30247649

RESUMO

Objectives: SSc is an autoimmune disease characterized by alteration of the immune response, vasculopathy and fibrosis. Most genetic studies on SSc have been performed in European-ancestry populations. The aim of this study was to analyse the genetic component of SSc in Middle Eastern patients from Iran and Turkey through a genome-wide association study. Methods: This study analysed data from a total of 834 patients diagnosed with SSc and 1455 healthy controls from Iran and Turkey. DNA was genotyped using high-throughput genotyping platforms. The data generated were imputed using the Michigan Imputation Server, and the Haplotype Reference Consortium as a reference panel. A meta-analysis combining both case-control sets was conducted by the inverse variance method. Results: The highest peak of association belonged to the HLA region in both the Iranian and Turkish populations. Strong and independent associations between the classical alleles HLA-DRB1*11: 04 [P = 2.10 × 10-24, odds ratio (OR) = 3.14] and DPB1*13: 01 (P = 5.37 × 10-14, OR = 5.75) and SSc were observed in the Iranian population. HLA-DRB1*11: 04 (P = 4.90 × 10-11, OR = 2.93) was the only independent signal associated in the Turkish cohort. An omnibus test yielded HLA-DRB1 58 and HLA-DPB1 76 as relevant amino acid positions for this disease. Concerning the meta-analysis, we also identified two associations close to the genome-wide significance level outside the HLA region, corresponding to IRF5-TNPO3 rs17424921-C (P = 1.34 × 10-7, OR = 1.68) and NFKB1 rs4648133-C (P = 3.11 × 10-7, OR = 1.47). Conclusion: We identified significant associations in the HLA region and suggestive associations in IRF5-TNPO3 and NFKB1 loci in Iranian and Turkish patients affected by SSc through a genome-wide association study and an extensive HLA analysis.


Assuntos
Escleroderma Sistêmico/genética , Alelos , Estudos de Casos e Controles , Predisposição Genética para Doença , Estudo de Associação Genômica Ampla , Genótipo , Cadeias beta de HLA-DP/genética , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Teste de Histocompatibilidade/métodos , Humanos , Fatores Reguladores de Interferon/genética , Irã (Geográfico)/epidemiologia , Polimorfismo Genético , Escleroderma Sistêmico/etnologia , Turquia/epidemiologia
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