Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 23
Filtrar
Mais filtros










Base de dados
Intervalo de ano de publicação
1.
J Rheumatol ; 44(5): 619-625, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28298568

RESUMO

OBJECTIVE: Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE). METHODS: For the study, 719 patients (76.9%) with aSLE and 216 (23.1%) with jSLE were examined. Comparisons between the groups were made for demographic characteristics, clinical features, auto-antibody profiles, damage, and survival rates. RESULTS: These results were significantly more frequent in jSLE: photosensitivity, malar rash, oral ulcers, renal involvement, neuropsychiatric (NP) manifestations, and autoimmune hemolytic anemia (AIHA). Of the autoantibodies, a higher frequency of anti-dsDNA and anticardiolipin IgG and IgM were observed in the jSLE group. A significant proportion of patients with aSLE had anti-Sm positivity and pleuritis. The proportion of patients with jSLE who developed organ damage was comparable to that of patients with aSLE (53% vs 47%) and the mean damage scores were similar in both groups. Renal damage was significantly more frequent in jSLE while musculoskeletal and cardiovascular system damage and diabetes mellitus were more prominent in aSLE. Comparison of survival rates of the 2 groups did not reveal any significant differences. CONCLUSION: We report a higher frequency in the jSLE group of renal involvement, cutaneous symptoms, oral ulcers, NP manifestations, AIHA, and anti-dsDNA positivity. A significant proportion of patients in the jSLE group had damage, most prominently in the renal domain. Our findings might support different genetic/environmental backgrounds for these 2 subgroups.


Assuntos
Autoanticorpos/imunologia , Rim/patologia , Lúpus Eritematoso Sistêmico/diagnóstico , Adolescente , Adulto , Fatores Etários , Idade de Início , Idoso , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/patologia , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida , Turquia/epidemiologia , Adulto Jovem
2.
Rheumatol Int ; 37(5): 675-684, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28289872

RESUMO

Patients with primary Sjogren's syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not. This is a multicenter study in which 834 patients from 22 university-based rheumatology clinics are included. Diagnosis of pSS was made on the clinical basis by the expert opinion. In this study, we only interviewed patients once and collected available data from the medical records. The European criteria, American-European Consensus Group (AECG) and American College of Rheumatology (ACR) Sjogren's criteria were applied. Majority of the patients were women (F/M was 20/1). The median duration from the first pSS-related symptom to diagnosis was significantly shorter in men (2.5 ± 2.3 vs 4.3 ± 5.9 years) (p = 0 < 0.016). When the European, AECG and ACR Sjogren's criteria were applied, 666 patients (79.9%) satisfied at least one of them. In total, 539 patients (64.4%) satisfied the European, 439 (52.6%) satisfied the AECG, and 359 (43%) satisfied the ACR criteria. Among the entire group, 250 patients (29.9%) satisfied all and 168 (20.1%) met none of the criteria. The rates of extraglandular organ involvements were not different between patients who met at least one of the criteria sets and those who met none. There is an urgent need for the modification of the pSS criteria sets to prevent exclusion of patients with extraglandular involvements as the dominant clinical features.


Assuntos
Síndrome de Sjogren/diagnóstico , Avaliação de Sintomas , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reumatologia , Adulto Jovem
3.
Clin Exp Rheumatol ; 35 Suppl 103(1): 77-82, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-27908308

RESUMO

OBJECTIVES: We aimed to assess the outcome of a large Takayasu arteritis (TAK) cohort using the vasculitis damage index (VDI) and quality of life (QoL) scale, tools which have been validated for vasculitis. METHODS: Disease activity, damage and QoL were cross-sectionally evaluated in 165 TAK patients from 6 centres. SF-36 were applied to 51 age-matched healthy controls (HC). Persistent activity for ≥6 months was considered as treatment resistance (r-TAK). The correlation between VDI, clinical characteristics and mental (MCS)/physical (PCS) component scores of SF-36 were analysed. SF-36 and VDI scores were compared between TAK subgroups and HC. RESULTS: The median age, follow-up time and disease duration were 40 (17-68), 60 (6-384), and 72 (6-396) months, respectively. 35% of them were r-TAK. VDI scores (VDIs) in TAK 4 (1-12) were mainly due to the disease itself [4 (1-10)]. VDIs in r-TAK were significantly higher than nr-TAK [5 (2-12) vs. 3 (2-10), p<0.001)]. In the TAK patients, MCS and PCS were found as 43±10 and 38±11, respectively. A high proportion of poor MCS (70%) and PCS (80%) were demonstrated in TAK. A significantly negative but weak correlation was observed between VDI and MCS (p=0.003, r=-0.23), PCS (p<0.001, r=-0.34). Higher VDIs were detected in patients with PCS <50 [5 (1-12) vs. 2 (1-6) p<0.001)]. SF-36 score was significantly lower in TAK than HC. CONCLUSIONS: Disease-related damage mainly caused by peripheral vascular involvement was more predominant than treatment-related damage without reaching the level of severe damage scores, but contributing to poor QoL, in the TAK cohort.


Assuntos
Qualidade de Vida , Arterite de Takayasu/patologia , Arterite de Takayasu/psicologia , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Estudos Transversais , Ciclofosfamida/uso terapêutico , Progressão da Doença , Resistência a Medicamentos , Feminino , Glucocorticoides/uso terapêutico , Nível de Saúde , Humanos , Imunossupressores/uso terapêutico , Masculino , Saúde Mental , Pessoa de Meia-Idade , Fatores de Risco , Índice de Gravidade de Doença , Arterite de Takayasu/tratamento farmacológico , Fatores de Tempo , Resultado do Tratamento , Turquia , Adulto Jovem
4.
Clin Exp Rheumatol ; 34 Suppl 100(5): 110-114, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27749243

RESUMO

OBJECTIVES: To determine the relationship between vascular biomarkers reflecting the vascular injury and organ involvement in systemic sclerosis (SSc). METHODS: Seventy-two SSc patients (66 female) fulfilling 2013 ACR/EULAR Criteria were evaluated. Serum samples of patients were collected for flow-cytometric analysis of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-ß1 and VCAM levels (Bender MedSystems) in SSc patients and 20 healthy controls. Results were compared with Pearson chi-square/Fisher's and Mann Whitney tests. RESULTS: Levels of MCP-1 were found to be elevated in patients with diffuse cutaneous SSc, flexion contractures, FVC<80%, DLCO<80%, pulmonary fibrosis and high acute phase response (p=0.002, p=0.005, p=0.045, p=0.005, p=0.036, p=0.006, respectively), TGF-ß1 in patients receiving immunosupressives (p=0.001), sE-selectin in patients with high acute phase response (p=0.028), sCD40L in patients with lcSSc (p=0.011) and smoking habitus (p=0.032). MCP-1 and sE-selectin levels were correlated with disease activity score (r=0.243, p=0.040 and r=0.303, p=0.010), MCP-1 and TGF-ß1 were correlated with severity of pulmonary involvement (r=0.323, p=0.006 and r=0.312, p=0.008). CONCLUSIONS: MCP-1 was the prominent biomarker correlated with the manifestations related to fibrosis, disease activity score and severity of pulmonary involvement. Treatment and smoking may have an effect on cytokine profile. Vascular biomarkers can be used to predict the characteristics and severity of SSc warranting prospective studies.


Assuntos
Quimiocina CCL2/sangue , Fibrose Pulmonar/sangue , Esclerodermia Difusa/sangue , Escleroderma Sistêmico/sangue , Pele/patologia , Tendões/patologia , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Estudos Transversais , Feminino , Fibrose , Citometria de Fluxo , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/etiologia , Fibrose Pulmonar/patologia , Fatores de Risco , Esclerodermia Difusa/complicações , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Difusa/patologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/patologia , Índice de Gravidade de Doença , Fumar/efeitos adversos , Regulação para Cima
5.
Microvasc Res ; 108: 17-21, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27392528

RESUMO

OBJECTIVE: To determine the relationship between vascular biomarkers reflecting the vascular injury and neoangiogenesis with capillaroscopic changes in systemic sclerosis (SSc). METHODS: Nailfold video-capillaroscopy (NVC) was performed qualitatively (early, active and late scleroderma patterns) in 72 SSc patients (66 female) fulfilling ACR/EULAR (2013) criteria. Serum samples of patients were collected and analysed by flow cytometer with multiplex kits of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-ß1 and VCAM at the same time with NVC. RESULTS: Compared to healthy subjects; tPA (p=0.02), MCP-1 (p=0.001), sE-selectin (p=0.008) and TGF-ß1 (p=0.001) levels were significantly higher, however sP-selectin (p=0.011) and IL-8 (p=0.001) levels were lower in SSc patients. SSc patients were defined according to NVC patterns as 'early' (n=10), 'active' (n=37) and 'late' (n=25). According to NVC patterns of SSc patients, only sCD40L levels were significantly lower in the 'late' group (p=0.039). The other markers were similar among NVC groups. CONCLUSIONS: NVC is a useful method for investigating the vascular pathogenesis and severity of SSc. Although the levels were similar to healthy controls in patients with early/active NVC patterns, there were lower sCD40L serum levels in patients with late NVC pattern. CD40L may have a role in the early/active phase of vascular involvement.


Assuntos
Ligante de CD40/sangue , Capilares/patologia , Angioscopia Microscópica , Unhas/irrigação sanguínea , Escleroderma Sistêmico/diagnóstico , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , Progressão da Doença , Feminino , Citometria de Fluxo , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/patologia , Índice de Gravidade de Doença
6.
Clin Exp Rheumatol ; 33(4 Suppl 91): S92-7, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25797836

RESUMO

OBJECTIVES: To determine the association of nailfold video-capillaroscopy (NVC) findings and telangiectasia score with digital ulcer (DU) history and severity of peripheral vascular involvement (PVI) in systemic sclerosis (SSc). METHODS: Fifty-nine SSc patients fulfilling Leroy & Medsger criteria were evaluated including telangiectasia score, disease activity and severity scores. NVC was performed according to qualitative (early, active and late patterns) and semi-quantitative assessments. RESULTS: When DU+ and DU- groups were compared; the mean score of capillary number (CN) was 2.0±0.5 vs. 1.4±0.7 (p<0.001), irregularly enlarged capillaries (IEC) was 1.8±0.6 vs. 1.4±0.7 (p<0.05), microangiopathy evolution score (MES) was 2.5±1.5 vs. 1.8±1.0 (p<0.05) and 'early' pattern was significantly less frequent in DU+ patients (1 vs. 9, p=0.016). The frequency of severe-PVI (Medsger severity score of 2-4) was 22% in females (12/54) and 80% in males (4/5). When severe and non-severe groups were compared; the mean score of CN was 2.1±0.4 vs. 1.5±0.7 (p<0.001), MES was 2.8±1.6 vs. 1.8±1.1 (p<0.05) and 'early' pattern was significantly less frequent in patients with severe PVI (0 vs. 9, p=0.049). The mean values of telangiectasia score were similar between groups. CONCLUSIONS: DU history and severe PVI in SSc were associated with capillary loss and microangiopathy. 'Early' NVC pattern was very rare in patients with DU history and was not found in severe PVI. Severe PVI in males was more frequent than females. Telangiectasia scores were not found to be related to PVI. NVC may be a helpful method in the assessment of SSc patients for PVI prognosis, warranting prospective studies.


Assuntos
Capilares/patologia , Dedos/irrigação sanguínea , Isquemia/diagnóstico , Angioscopia Microscópica , Unhas/irrigação sanguínea , Doenças Vasculares Periféricas/diagnóstico , Escleroderma Sistêmico/complicações , Úlcera Cutânea/diagnóstico , Telangiectasia/diagnóstico , Adulto , Estudos Transversais , Feminino , Humanos , Isquemia/etiologia , Isquemia/patologia , Masculino , Angioscopia Microscópica/métodos , Pessoa de Meia-Idade , Doenças Vasculares Periféricas/etiologia , Doenças Vasculares Periféricas/patologia , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Índice de Gravidade de Doença , Fatores Sexuais , Úlcera Cutânea/etiologia , Úlcera Cutânea/patologia , Telangiectasia/etiologia , Telangiectasia/patologia , Gravação em Vídeo
7.
J Rheumatol ; 41(7): 1304-10, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24833757

RESUMO

OBJECTIVE: Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE. METHODS: We analyzed 852 patients with SLE who attended our clinic. Seven autoantibodies were selected for cluster analysis: anti-DNA, anti-Sm, anti-RNP, anticardiolipin (aCL) immunoglobulin (Ig)G or IgM, lupus anticoagulant (LAC), anti-Ro, and anti-La. Two-step clustering and Kaplan-Meier survival analyses were used. RESULTS: Five clusters were identified. A cluster consisted of patients with only anti-dsDNA antibodies, a cluster of anti-Sm and anti-RNP, a cluster of aCL IgG/M and LAC, and a cluster of anti-Ro and anti-La antibodies. Analysis revealed 1 more cluster that consisted of patients who did not belong to any of the clusters formed by antibodies chosen for cluster analysis. Sm/RNP cluster had significantly higher incidence of pulmonary hypertension and Raynaud phenomenon. DsDNA cluster had the highest incidence of renal involvement. In the aCL/LAC cluster, there were significantly more patients with neuropsychiatric involvement, antiphospholipid syndrome, autoimmune hemolytic anemia, and thrombocytopenia. According to the Systemic Lupus International Collaborating Clinics damage index, the highest frequency of damage was in the aCL/LAC cluster. Comparison of 10 and 20 years survival showed reduced survival in the aCL/LAC cluster. CONCLUSION: This study supports the existence of autoantibody clusters with distinct clinical features in SLE and shows that forming clinical subsets according to autoantibody clusters may be useful in predicting the outcome of the disease. Autoantibody clusters in SLE may exhibit differences according to the clinical setting or population.


Assuntos
Autoanticorpos/sangue , Lúpus Eritematoso Sistêmico/imunologia , Adulto , Autoanticorpos/imunologia , Análise por Conglomerados , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Masculino , Pessoa de Meia-Idade , Adulto Jovem
8.
Clin Rheumatol ; 31(2): 325-8, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21814755

RESUMO

Recently, a new classification algorithm (CA) for systemic necrotizing vasculitides was proposed by Watts et al. (Annals Rheum Dis 66:222-227, 2007) by using the American College of Rheumatology (ACR), Chapel Hill Consensus Criteria (CHCC) and Sorensen surrogate markers (So). We aimed to validate CA in our patients. One hundred twenty-nine patients followed up in our vasculitis clinic were reclassified according to CA in different categories (ACR or Lanham criteria in "1" for Churg-Strauss Syndrome (CSS); ACR in "2a"; CHCC-Wegener's granulomatosis (WG) in "2b"; CHCC-microscopic polyangiitis (MPA), So-WG in "2c"; So-WG, proteinase 3 (PR3) or myeloperoxidase antineutrophil cytoplasmic antibody (MPO ANCA) serology in "2d" for WG; clinical features and histology compatible with small vessel vasculitis without So-WG in "3a"; So-MPA, PR3 or MPO ANCA serology in "3b" for MPA; CHCC-classic-polyarteritis nodosa (c-PAN) or typical angiographic features in "4" for c-PAN; unclassifiable in "5"). Kappa statistic was used to analyse the agreement of the criteria that formed the algorithm. All of 12 CSS, 91% of 69 WG, 78% of 18 MPA and 93% of 26 c-PAN patients remained in their previous diagnosis. WG patients were placed in 2a (83%), 2c (3%), 2d (14%) categories. Four WG (6%) and four MPA (22%) patients were categorized as MPA (in 3a (75%), 3b (25%)) and WG (in 2c (75%), 2d (25%)), respectively. Three of four unclassified patients could be classified as c-PAN (two) and MPA (one). Significant agreement was demonstrated only for ACR and So criteria in WG (κ = 0.62, p < 0.001). The majority of our patients stayed on their previous diagnosis in "CA". Our findings suggest that this algorithm is helpful and practical for epidemiological studies. Poor correlation of defined criteria was thought to be related to the fact that each criteria mainly consist of different characteristics of vasculitides such as clinical, histopathological and serological features.


Assuntos
Vasculite Sistêmica/classificação , Adulto , Algoritmos , Humanos , Vasculite Sistêmica/diagnóstico
9.
Rheumatol Int ; 32(1): 79-83, 2012 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20661740

RESUMO

Previous studies showed a link between systemic lupus erythematosus (SLE) and Epstein-Barr virus (EBV) infection. We sought to determine the features of serologic response to EBV in SLE patients and whether this response differs from those of systemic sclerosis (SSc) and primary antiphospholipid syndrome (PAPS) patients as well as healthy individuals. Sera from 198 consecutive SLE patients have been tested to detect IgG antibodies to EA/D, EBNA-1, VCA P18 and for comparison, cytomegalovirus (CMV) using commercially available ELISA kits (Trinity Biotech, USA). Forty-six SSc patients and 38 PAPS patients were enrolled as diseased control groups and sixty-five individuals as healthy controls. Significantly more SLE (54%, P = 0.001, OR 5.77, 95% CI 2.8-11.6), SSc (41.3%, P = 0.005, OR 3.4, 95% CI 1.4-8.2) and PAPS sera (36.8%, P = 0.023, OR 2.86, 95% CI 1.14-7.22) reacted against EA/D than healthy controls (16.9%). The mean age of anti-EA/D-positive SLE patients was significantly higher, and their disease duration was longer compared to anti-EA/D-negative SLE patients (41 ± 14 vs. 33.8 ± 10.8 years, P < 0.001 and 100 ± 73 vs. 71 ± 62 months, P = 0.003). In SLE patients, EA/D reactivity was associated with Raynaud's phenomenon and the presence of any anti-ENA antibodies. Although it did not reach a statistical significance, anti-EBNA-1 reactivity was slightly lower in patients with SLE. The frequency of anti-CMV Ig G positivity was found significantly higher in SLE patients (100%) when compared to patients with SSc (95.7%), PAPS (94.7%) and healthy controls (95.4%) (P = 0.035, P = 0.025 and P = 0.015 respectively). Our results support the proposed link between EBV and SLE. The finding that SSc and PAPS patients also have increased frequency of anti-EA/D response has revealed that this immune interaction may not be unique to patients with SLE, and there may be a common mechanism involving EBV in these autoimmune diseases.


Assuntos
Anticorpos Antivirais/sangue , Antígenos Virais/imunologia , Síndrome Antifosfolipídica/imunologia , Herpesvirus Humano 4/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Escleroderma Sistêmico/imunologia , Adolescente , Adulto , Idoso , Síndrome Antifosfolipídica/sangue , Proteínas do Capsídeo/imunologia , Estudos de Casos e Controles , Citomegalovirus/imunologia , Antígenos Nucleares do Vírus Epstein-Barr/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/sangue , Estudos Soroepidemiológicos , Adulto Jovem
10.
Rheumatol Int ; 32(1): 177-82, 2012 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20711782

RESUMO

The objective was to investigate the predictive factors for avascular necrosis (AVN) of bone in patients with systemic lupus erythematosus (SLE). The records of 868 patients with SLE from four centers were reviewed retrospectively. Forty-nine patients with AVN were identified. A total of 154 patients with SLE who did not have clinically apparent AVN during the follow-up were evaluated as a control group. The demographic, clinical, laboratory and management characteristics of these two groups of patients were recorded according to predefined protocol and compared. The prevalence of AVN was detected 6% in our SLE population. The highest dose corticosteroid administered within 4 months and total cumulative prednisolone dose were significantly higher in the SLE patients with AVN. The use of cytotoxic agent significantly higher proportion of patients with AVN. AVN tends to develop more frequently in male gender and younger patients. Oral ulcer, pleuritis, Raynaud's phenomenon, cutaneous vasculitis, lymphadenopathy, autoimmune thyroiditis, peripheral neuropathy and Sjögren's syndrome were higher incidence in SLE patients with AVN. The bilateral femoral heads were the commonest site of involvement of AVN in our patients with SLE.


Assuntos
Necrose da Cabeça do Fêmur/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Osteonecrose/epidemiologia , Adolescente , Corticosteroides/uso terapêutico , Adulto , Fatores Etários , Estudos de Casos e Controles , Relação Dose-Resposta a Droga , Feminino , Humanos , Masculino , Prednisolona/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Adulto Jovem
11.
Rheumatol Int ; 31(2): 183-9, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20012052

RESUMO

Pulmonary hypertension (PH) in systemic lupus erythematosus (SLE) is associated with an unfavorable prognosis. We investigated the characteristics of SLE patients with PH. The patients with a pulmonary artery systolic pressure more than 30 mmHg at rest on echocardiogram were diagnosed with PH. Echocardiography was done only in patients with clinical or radiological evidence suggesting PH. Right heart catheterization was not performed. We identified 10 SLE patients with PH between 1980 and 2000. We compared their clinical and laboratory parameters with those of 97 consecutive SLE patients without PH. Nine of the ten patients with PH were females. The mean age at the time of SLE onset was 25.2 ± 6.9 years; the mean duration of follow-up was 93.4 ± 52.8 months, and the interval between the onset of SLE and PH diagnosis was 9.0 ± 4.6 (5-21) years. Antiphospholipid antibody positivity was significantly higher in the PH group (80 vs. 36%; p < 0.05), but there was no significant difference between two groups in regard to secondary antiphospholipid syndrome. The frequency of Raynaud's phenomenon was higher in PH group (60 vs. 27%; p < 0.05). Renal involvement (80 vs. 43%; p < 0.05), neuropsychiatric involvement (40 vs. 7.2%; p < 0.005) and serositis (70 vs. 14.4%; p < 0.001) were significantly more frequent in the PH group. The mean damage score in patients with and without PH were 4.0 ± 2.4 and 0.4 ± 1.0, respectively (p < 0.001). Four patients with PH died during the follow-up. This study reveals that the presence of PH defines a subgroup of patients with a severe disease and increased mortality. Antiphospholipid antibodies and Raynaud's phenomenon may contribute to the pathogenesis of PH. We recommend that all patients with SLE, especially those positive for antiphospholipid antibodies and/or with signs of Raynaud's phenomenon should be regularly evaluated for the development of PH.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Hipertensão Pulmonar/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Adulto , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Nefropatias/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Transtornos Mentais/epidemiologia , Prognóstico , Doença de Raynaud/epidemiologia , Estudos Retrospectivos , Serosite/epidemiologia , Índice de Gravidade de Doença , Resultado do Tratamento , Ultrassonografia , Adulto Jovem
12.
Arthritis Rheum ; 62(11): 3232-6, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20669279

RESUMO

OBJECTIVE: The pathogenesis of ankylosing spondylitis (AS) has a strong genetic contribution. Familial Mediterranean fever (FMF) is an autosomal recessively inherited autoinflammatory disorder caused by MEFV gene missense variations, and a clinical association between FMF and AS has been reported previously. The aim of this study was to analyze the association of common MEFV variations (M694V, M680I, V726A, and E148Q) with AS in a group of Turkish patients. METHODS: The study group comprised 193 patients with AS and 103 matched healthy control subjects. All individuals were genotyped for 4 MEFV variations and HLA-B27 using genomic DNA, and association of the variations with the clinical and laboratory features of the patients was analyzed. RESULTS: The MEFV missense variations were significantly more frequent in patients with AS (22.3%) compared with healthy control subjects (9.7%; odds ratio [OR] 2.67, 95% confidence interval [95% CI] 1.28-5.56). This difference was more prominent for exon 10 variations (M694V, V726A, M680I) (OR 3.75, 95% CI 1.41-9.97), especially for the most-penetrant variation M694V (OR 4.73, 95% CI 1.39-16.12). MEFV variations were more frequent in HLA-B27-negative patients with AS, and the difference was statistically significant in patients carrying exon 10 variants. CONCLUSION: FMF-related MEFV variations are associated with AS, and these variations may contribute to the pathogenesis of AS, especially in populations in which the prevalence of FMF is high.


Assuntos
Proteínas do Citoesqueleto/genética , Espondilite Anquilosante/genética , Adolescente , Adulto , Idoso , Alelos , Feminino , Frequência do Gene , Estudos de Associação Genética , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto , Razão de Chances , Pirina , Turquia
13.
J Rheumatol ; 37(2): 374-8, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20008921

RESUMO

OBJECTIVE: To evaluate damage features and impact on survival by Vasculitis Damage Index (VDI) in a cohort of Turkish patients with Wegener's granulomatosis (WG). METHODS: We enrolled 50 (25 female) patients with WG according to ACR criteria. Birmingham Vasculitis Activity Score (BVAS) and VDI were used to analyze disease activity and damage. RESULTS: Patients had kidney (82%), upper airway (72%), lung (70%), and nervous system (15%) involvement. Median age at diagnosis was 45 years, time to diagnosis was 3.5 months, and total followup time was 35.5 months. All but one patient was positive for antineutrophil cytoplasmic antibodies (ANCA). Mean final dose and duration of corticosteroid and cyclophosphamide was 15 +/- 14 g, 39 +/- 33 months and 36 +/- 34 g, 21 +/- 2 months, respectively. Mean early (e) BVAS were 20.2 +/- 7.1 (4-38) (median 21). Mean e-BVAS and e-VDI scores at presentation and final (f)-VDI scores at last visit were 20.2 +/- 7.1 (4-38), 3.1 +/- 1.7 (median 3) (0-7) and 4.4 +/- 2.6 (0-12), consecutively. Disease related damage was prominent in kidneys (50%) and upper airways (27%). Amenorrhea (90%), cataract (28%), and diabetes (24%) were the most frequent treatment related damages. Rapidly progressive glomerulonephritis at presentation (42%) progressed to endstage renal failure in 20%. Relapses occurred in 25% with mean BVAS of 6.5 +/- 2.3 (4-11). Survival rate was 77% at 37 months. Deaths occurred early (90% in the first year). f-VDI was high in patients who relapsed (6 +/- 3 vs 3.8 +/- 2.1, p = 0.03). Logistic regression analysis demonstrated that age at time of diagnosis and e-VDI were lower in survivors with OR = 0.9 (p = 0.06, 95% CI: 0.78-1) and OR = 0.5 (p = 0.04, 95%CI: 0.25-0.98), respectively. In this cohort, e-VDI score of 5 or more was related to death with 98% sensitivity and 56% specificity (p = 0.004) (CI: 0.66-0.95). CONCLUSION: Disease related damage outweighed treatment related damage in our cohort of predominantly generalized disease activity. Early damage and older age were found to be predictors of final damage and death.


Assuntos
Granulomatose com Poliangiite/mortalidade , Vasculite/mortalidade , Adolescente , Adulto , Idoso , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Ciclofosfamida/uso terapêutico , Ensaio de Imunoadsorção Enzimática , Feminino , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Falência Renal Crônica/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Análise de Regressão , Índice de Gravidade de Doença , Estatísticas não Paramétricas , Taxa de Sobrevida , Vasculite/complicações , Vasculite/tratamento farmacológico
14.
Clin Rheumatol ; 28(6): 733-6, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-19326165

RESUMO

A 17-year-old female patient presented with chronic symmetrical oligoarthritis of both knees and ankles, xerostomia, xerophthalmia, multiple bilateral lymphadenopathies in the cervical region, and bilateral parotid enlargement with the histological finding of chronic sialoadenitis. She had been already given methotrexate, chloroquine, and corticosteroids with the diagnosis of rheumatoid arthritis (RA) before referral to our outpatient clinic. Because her complaints and the lumps did not remit and she could be classified as neither RA nor primary Sjögren's syndrome (SS) according to 1987 ACR RA criteria or European preliminary criteria for SS, lymph node biopsy was repeated and revealed the diagnosis of Rosai-Dorfman disease (RDD) with the histological findings of histiocytes, phagocyting lymphocytes in enlarged sinuses, and mature plasma cells infiltrating the pulpa. All the medications were stopped after the pathological diagnosis of RDD and consulting with the Division of Hematology. She was reevaluated with magnetic resonance imaging, which showed dense infiltrative areas around knee and ankle joints, and computed tomography that showed a soft tissue mass surrounding the descending aorta and upper part of the abdominal aorta. Activated partial thromboplastin time was found to be prolonged in prebiopsy examinations, and factor XII deficiency was detected after detailed hematological evaluation. The symptoms of joint involvement were relieved with nonsteroidal antiinflammatory drugs. She has been followed-up without medication without obvious clinical or laboratory change. We herein report a patient with RDD mimicking RA and SS. We consider that RDD should be kept in mind especially in patients with resistant symptoms to conventional therapies, younger disease onset, and predominant parotid and lymph node enlargement.


Assuntos
Deficiência do Fator XII/complicações , Deficiência do Fator XII/diagnóstico , Histiocitose Sinusal/complicações , Histiocitose Sinusal/diagnóstico , Adolescente , Articulação do Tornozelo/diagnóstico por imagem , Articulação do Tornozelo/patologia , Artrite Reumatoide/diagnóstico , Diagnóstico Diferencial , Deficiência do Fator XII/patologia , Feminino , Histiocitose Sinusal/patologia , Humanos , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/patologia , Imagem por Ressonância Magnética , Síndrome de Sjogren/diagnóstico , Tomografia Computadorizada por Raios X
15.
Rheumatol Int ; 29(4): 365-70, 2009 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-18766345

RESUMO

Several criteria are being used for the classification of psoriatic arthritis (PsA) and there is a lack of consensus about PsA as a separate entity. Our aim is to investigate the clinical features of our patients with a clinical diagnosis of PsA, compare the sensitivities of different classification criteria, agreement between the criteria. In this study 86 PsA patients were investigated (48 females, mean age 44). Moll and Wright criteria was fulfilled by 91%, Vasey and Espinoza criteria by 94% and modified European SpA study group criteria by 59%, classification of PsA study group criteria by 86%, modified McGonagle criteria by 96%, Fournie et al. criteria by 84% and Gladman criteria by 95%. Significant agreement was present between criteria but generally kappa-values were less than 0.5. The pattern of PsA can differ with time and the implementation of the available classification criteria showed considerable differences.


Assuntos
Artrite Psoriásica/classificação , Artrite Psoriásica/diagnóstico , Espondiloartropatias/classificação , Espondiloartropatias/diagnóstico , Adolescente , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Cooperação Internacional , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Turquia , Adulto Jovem
16.
Joint Bone Spine ; 75(3): 299-302, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-18424159

RESUMO

OBJECTIVES: Ankylosing spondylitis is strongly associated with HLA-B27. However, the strength of the association with HLA-B27 and the clinical features may vary in different parts of the world. The aim of this study is to compare the clinical features of AS and the frequencies of HLA-B27 and its alleles in patients from Turkey with other series. METHODS: One hundred and twelve patients (72 male/40 female) fulfilling the modified New York criteria for the classification of AS and 55 (27 male/28 female) healthy controls were typed for HLA-B27 and allele frequencies by sequence specific primer (PCR/SSP) method and assessed for clinical manifestations. RESULTS: Male to female ratio was 1.8, mean age at disease onset was 23.5 and 24.1% of patients reported juvenile onset of symptoms. Peripheral arthritis was seen in 52.7% of patients. Family history (p=0.01) and peripheral arthritis (p=0.02) were more frequent in females and spinal involvement in males. HLA-B27 was found to be positive in 70% of patients and associated with younger mean age, uveitis and shorter time elapsed from symptom to diagnosis. The frequency of HLA-B27 alleles associated with SpA was not different between ankylosing spondylitis patients and healthy controls. CONCLUSION: Low frequency of HLA-B27 and clinical variations in ankylosing spondylitis may be due to different genetic and/or environmental factors in Turkey.


Assuntos
Antígeno HLA-B27/genética , Espondilite Anquilosante/genética , Feminino , Frequência do Gene , Humanos , Masculino , Turquia
17.
Rheumatol Int ; 28(10): 959-64, 2008 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-18317769

RESUMO

Several criteria are being used for the classification of psoriatic arthritis (PsA) and there is a lack of consensus about PsA as a separate entity. Our aim is to investigate the clinical features of our patients with a clinical diagnosis of PsA, compare the sensitivities of different classification criteria and agreement between the criteria. In this study 86 PsA patients were investigated (48 female, mean age 44). Moll and Wright criteria were fulfilled by 91%, Vasey and Espinoza criteria by 94% and modified European SpA study group criteria by 59%, classification of PsA study group criteria by 86%, modified McGonagle criteria by 96%, Fournié et al. criteria by 84%, and Gladman criteria by 95%. Significant agreement was present between criteria but generally kappa values were less than 0.5. The pattern of PsA can differ with time and the implementation of the available classification criteria showed considerable differences.


Assuntos
Artrite Psoriásica/classificação , Artrite Psoriásica/diagnóstico , Espondiloartropatias/classificação , Espondiloartropatias/diagnóstico , Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Turquia , Adulto Jovem
18.
Clin Rheumatol ; 26(11): 1913-5, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17636363

RESUMO

It is reported that the usage of high-dose intravenous immunoglobulin (HD-IVIG) in systemic autoimmune diseases is associated with various adverse events in a wide range of severity. We aimed to investigate the frequency and profile of adverse events in a group of patients with diffuse connective tissue diseases and Wegener's granulomatosis (WG) who were administrated HD-IVIG for different indications. We recorded the data of 38 patients (25 females and 13 males) aged 38 +/- 15 (12-75) years who were followed up with the diagnosis of systemic autoimmune diseases between 1994 and 2006 according to a predefined protocol. Patients with active disease were treated with HD-IVIG and standard immunosuppressives concomitantly. We evaluated the occurrence of allergy, acute renal failure, thromboembolic events, neutropenia, hemolytic anemia, aseptic meningitis, and vasculitis during infusion therapy of HD-IVIG and in the following 3 weeks. We commenced a total of 130 infusions of HD-IVIG. Patients were administrated 1-12 (3.4 +/- 2.6) infusions of HD-IVIG as needed. Indications for HD-IVIG were unresponsiveness or partial response to standard treatment, severe infections along with disease activity, and severe thrombocytopenia in the preoperative period in 97, 23, and 5% of patients, respectively. Minor adverse events were seen in two patients during HD-IVIG infusions. One patient with WG developed rapidly progressive renal failure during severe disease flare between HD-IVIG infusions. Another patient with WG developed recurrence of deep-vein thrombosis during severe disease flare 3 months after HD-IVIG. Both events were attributed to severe disease activity. Adverse events like allergy, acute renal failure, thromboembolic events, hematological problems, aseptic meningitis, and vasculitis are reported in different frequencies (1-81%) in patients who were administered HD-IVIG for systemic autoimmune diseases. HD-IVIG is considered a safe treatment in selected patients assuring adequate infusion precautions.


Assuntos
Imunoglobulinas Intravenosas/efeitos adversos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/efeitos adversos , Fatores Imunológicos/uso terapêutico , Adolescente , Adulto , Idoso , Doenças Autoimunes/imunologia , Doenças Autoimunes/terapia , Criança , Feminino , Humanos , Síndromes de Imunodeficiência/imunologia , Síndromes de Imunodeficiência/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Segurança , Resultado do Tratamento
19.
Clin Rheumatol ; 24(6): 673-6, 2005 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-15926038

RESUMO

The objective of this study was to investigate the frequency of antibodies against cyclic citrullinated peptides (anti-CCP) and keratin (AKA) in patients with rheumatoid arthritis (RA) as well as in patients with primary Sjögren's syndrome (pSS) and Wegener's granulomatosis (WG), who may present with rheumatoid factor (RF)-positive arthritis. The study group consisted of 46 patients with RA (26 patients were negative for RF), 32 with pSS, 22 with WG, and 40 healthy controls. The RF, anti-CCP, and AKA were detected in serum using the latex agglutination test, enzyme-linked immunosorbent assay, and indirect immunofluorescence, respectively. The agreement between those tests was evaluated by kappa test. No positive result for AKA was detected in pSS and WG patients, and anti-CCP was found in only one patient with pSS. The results of kappa tests were low, varying between 0.25 (RF-anti-CCP) and 0.02 (RF-AKA). The sensitivity and specificity values were 43 and 44% for RF, 65 and 98% for anti-CCP, and 58 and 100% for AKA, respectively, in RA patients. In the RF-negative RA group, AKA was found to have a high frequency (55%) in comparison to anti-CCP (38%). Seropositivity was found to be 87% for any one of the three autoantibodies tested in RA patients. With a higher specificity, values for RA, anti-CCP, and AKA seem to be helpful for the differential diagnosis of patients with RF-positive arthritis, which may include patients with WG and pSS, and screening of all three antibodies may increase the diagnostic performance.


Assuntos
Artrite Reumatoide/imunologia , Autoanticorpos/sangue , Granulomatose com Poliangiite/imunologia , Queratinas/imunologia , Peptídeos Cíclicos/imunologia , Síndrome de Sjogren/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Artrite Reumatoide/diagnóstico , Biomarcadores/sangue , Feminino , Granulomatose com Poliangiite/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Fator Reumatoide/sangue , Sensibilidade e Especificidade , Síndrome de Sjogren/diagnóstico
20.
Rheumatol Int ; 25(3): 211-4, 2005 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-14722731

RESUMO

We aimed to investigate the efficacy and safety profile of high-dose intravenous immunoglobulin (HD-IVIG) therapy in patients with severe systemic lupus erythematosus (SLE), inflammatory muscle disease (IMD), Wegener's granulomatosis (WG), and/or concurrent infection who failed to respond to standard therapies. We evaluated the records of eight patients with SLE, eight with IMD, and four with WG who were treated with HD-IVIG (2 g/kg per month for 1-12 months) for active disease in 19 patients and concurrent infection in three (mycobacterial in two with SLE and cytomegaloviral in one with WG). Systemic lupus erythematosus disease activity index (SLEDAI) scores before and after HD-IVIG were statistically analysed. Remission was achieved in 14 cases (70%). The SLEDAI scores significantly decreased in patients with SLE (P=0.02). No serious side effect was observed. High-dose IVIG may be used as an adjunctive treatment in connective tissue diseases that do not respond to standard therapies or as alternative treatment for patients with concurrent severe infections or for whom immunosuppressives are contraindicated.


Assuntos
Doenças do Tecido Conjuntivo/tratamento farmacológico , Doenças do Tecido Conjuntivo/patologia , Imunoglobulinas Intravenosas/administração & dosagem , Imunossupressores/administração & dosagem , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Estudos de Coortes , Doenças do Tecido Conjuntivo/mortalidade , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Seguimentos , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Miosite/diagnóstico , Miosite/tratamento farmacológico , Estudos Retrospectivos , Medição de Risco , Fatores Sexuais , Taxa de Sobrevida , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA