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1.
Rheumatol Int ; 39(6): 1061-1067, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30888471

RESUMO

Arterial aneurysms are rare manifestations of Behçet Disease (BD) with high morbidity and mortality. This study aimed to investigate the clinical course of BD patients with abdominal aortic aneurysms (AAA). We retrospectively searched charts of BD patients, followed up between 1988 and 2011, to identify those with AAA with at least 6-month clinical and radiological follow-up data. Chart review revealed 12 patients (11 males) with AAA amongst 1224 patients; follow-up data from 11 patients were available. The most common symptoms were lower back and abdominal pain. The only pre-treatment complication was a spontaneous rupture. All but one patient received corticosteroid and cyclophosphamide pulses for the induction, and corticosteroid and azathioprine for the maintenance treatment; one patient received only the maintenance treatment. Two patients had surgical graft interposition, without postoperative complications. Seven patients had endovascular stenting; five of them (71.4%) showed radiological regression after 32.5 (13.4-53.8) months, while four (57%) had clinical improvement after 11.8 (0.2-29.4) months. However, one non-responsive patient developed stent infection and exsanguinated during percutaneous drainage, and one patient developed femoral artery pseudo-aneurysm at the catheter insertion site. Another patient developed a new aneurysm under the maintenance treatment. Medical treatment alone yielded radiological regression in one of two patients. Current immunosuppressive, surgical or endovascular approaches can provide clinical and radiological improvements lately in BD patients with AAA. Furthermore, complication rates seem to be high with interventional approaches. These findings suggest an unmet need for safer alternative treatments.


Assuntos
Corticosteroides/uso terapêutico , Aneurisma da Aorta Abdominal/terapia , Azatioprina/uso terapêutico , Síndrome de Behçet/terapia , Ciclofosfamida/uso terapêutico , Procedimentos Endovasculares , Imunossupressores/uso terapêutico , Stents , Enxerto Vascular , Dor Abdominal , Adulto , Falso Aneurisma/epidemiologia , Aneurisma da Aorta Abdominal/etiologia , Ruptura Aórtica , Síndrome de Behçet/complicações , Feminino , Artéria Femoral , Humanos , Quimioterapia de Indução , Dor Lombar , Quimioterapia de Manutenção , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Infecções Relacionadas à Prótese/epidemiologia , Estudos Retrospectivos , Adulto Jovem
2.
Front Immunol ; 9: 2244, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30323817

RESUMO

Antiphospholipid antibodies (aPL), the serological hallmark of antiphospholipid syndrome (APS), are a heterogeneous group of autoantibodies raised against circulating blood proteins. Of these proteins, the phospholipid-binding b2-glycoprotein I (ß2GPI) is considered to be the main autoantigen in APS. Indeed, IgG antibodies targeting b2GPI (ab2GPI) directly cause both thrombosis and pregnancy morbidity in several mouse models. While antibodies raised against all five domains of b2GPI have been reported, a subgroup of IgG ab2GPI raised against the first domain (DI) of b2GPI (aDI), strongly correlate with thrombotic APS, and drive thrombosis and pregnancy loss in vivo. Few studies have focused on determining the type of IgG subclass(es) for aPL. The subclass of an antibody is important as this dictates the potential activity of an antibody; for example, IgG1 and IgG3 can fix complement better and are able to cross the placenta compared to IgG2 and IgG4. It is unknown what subclass IgG aDI are, and whether they are the same as ab2GPI. To determine IgG subclass distribution for ab2GPI and aDI, we purified total IgG from the serum of 19 APS patients with known ab2GPI and aDI activity. Using subclass-specific conjugated antibodies, we modified our established in-house ab2GPI and aDI ELISAs to individually measure IgG1, IgG2, IgG3, and IgG4. We found that while IgG1, IgG2, and IgG3 ab2GPI levels were similar, a marked difference was seen in IgG subclass aDI levels. Specifically, significantly higher levels of IgG3 aDI were detected compared to IgG1, IgG2, or IgG4 (p < 0.05 for all comparisons). Correlation analysis of subclass-specific ab2GPI vs. aDI demonstrated that IgG3 showed the weakest correlation (r = 0.45, p = 0.0023) compared to IgG1 (r = 0.61, p = 0.0001) and IgG2 (r = 0.81, p = 0.0001). Importantly, total subclass levels in IgG purified from APS and healthy serum (n = 10 HC n = 12 APS) did not differ, suggesting that the increased IgG3 aDI signal seen in APS-derived IgG is antigen-specific. To conclude, our data suggests that aDI show a different IgG subclass distribution to ab2GPI. Our results highlight the importance of aDI testing for patient stratification and may point toward differential underlying aPL-driven pathogenic processes that may be subclass restricted.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Ensaio de Imunoadsorção Enzimática/métodos , Imunoglobulina G/sangue , Domínios Proteicos/imunologia , beta 2-Glicoproteína I/sangue , Adulto , Análise de Variância , Feminino , Humanos , Imunoglobulina G/imunologia , Masculino , Pessoa de Meia-Idade , beta 2-Glicoproteína I/imunologia
3.
Sci Rep ; 7(1): 10788, 2017 09 07.
Artigo em Inglês | MEDLINE | ID: mdl-28883515

RESUMO

Factor (F) Xa reactive IgG isolated from patients with antiphospholipid syndrome (APS) display higher avidity binding to FXa with greater coagulant effects compared to systemic lupus erythematosus (SLE) non APS IgG. FXa signalling via activation of protease-activated receptors (PAR) leads to increased intracellular calcium (Ca2+). Therefore, we measured alterations in Ca2+ levels in human umbilical vein endothelial cells (HUVEC) following FXa-mediated PAR activation and investigated whether FXa reactive IgG from patients with APS or SLE/APS- alter these responses. We observed concentration-dependent induction of Ca2+ release by FXa that was potentiated by APS-IgG and SLE/APS- IgG compared to healthy control subjects' IgG, and FXa alone. APS-IgG and SLE/APS- IgG increased FXa mediated NFκB signalling and this effect was fully-retained in the affinity purified anti-FXa IgG sub-fraction. Antagonism of PAR-1 and PAR-2 reduced FXa-induced Ca2+ release. Treatment with a specific FXa inhibitor, hydroxychloroquine or fluvastatin significantly reduced FXa-induced and IgG-potentiated Ca2+ release. In conclusion, PAR-1 and PAR-2 are involved in FXa-mediated intracellular Ca2+ release in HUVEC and FXa reactive IgG from patients with APS and/or SLE potentiate this effect. Further work is required to explore the potential use of IgG FXa reactivity as a novel biomarker to stratify treatment with FXa inhibitors in these patients.


Assuntos
Síndrome Antifosfolipídica/imunologia , Síndrome Antifosfolipídica/metabolismo , Cálcio/metabolismo , Células Endoteliais/metabolismo , Fator Xa/metabolismo , Imunoglobulina G/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/metabolismo , Biomarcadores , Estudos de Casos e Controles , Feminino , Células Endoteliais da Veia Umbilical Humana , Humanos , Espaço Intracelular/metabolismo , Masculino , Receptor PAR-1/antagonistas & inibidores , Receptor PAR-2/antagonistas & inibidores , Transdução de Sinais/efeitos dos fármacos
4.
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
5.
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
6.
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
7.
Arthritis Res Ther ; 17: 47, 2015 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-25890027

RESUMO

INTRODUCTION: The aim of this study was to examine the prevalence and functional effects of antibodies directed against Factor (F)Xa and other serine proteases (SP) in patients with antiphospholipid syndrome (APS). METHODS: Serum from patients with APS (n=59), systemic lupus erythematosus (SLE; n=106), other autoimmune rheumatic disease (ARD; n=63) and 40 healthy controls (HC) were tested for IgG activity against thrombin (Thr), FXa, FVIIa, phosphatidylserine (PS)/FXa and antithrombin (AT)-III by enzyme-linked immunosorbent assay (ELISA). Anti-FXa positive IgG were purified to measure their avidity by chaotropic ELISA and functional effects upon clotting time (FXa-ACT) and FXa enzymatic activity (±AT-III). RESULTS: Anti-FXa IgG were found in patients with SLE (49.1%) and APS (33.9%) (P<0.05) but not in ARD controls and HC. In contrast, anti-Thr and anti-PS/FXa IgG were identified in other ARD and anti-FVIIa IgG were low in all groups. The avidity of APS-IgG to FXa was significantly higher than SLE-IgG (P<0.05). Greatest prolongation of FXa-ACT was observed with APS-IgG and greatest inhibitory effect upon FXa enzymatic activity was found with APS-IgG followed by SLE-IgG compared to HC-IgG. ATIII inhibition of FXa was significantly reduced by APS-IgG compared with HC and SLE (P<0.05) and did not correlate with binding to AT-III. CONCLUSION: APS anti-FXa IgG have higher avidity to FXa and greater effects upon the enzymatic and coagulant activity of FXa compared with SLE anti-FXa IgG. Further studies of anti-FXa antibodies in APS, SLE and other non-autoimmune thrombotic disease cohorts are now required to evaluate whether targeting FXa with selective inhibitors in patients bearing anti-FXa antibodies may be an effective treatment strategy.


Assuntos
Síndrome Antifosfolipídica/imunologia , Autoanticorpos/sangue , Coagulação Sanguínea , Fator Xa/imunologia , Adulto , Síndrome Antifosfolipídica/sangue , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
8.
Curr Rheumatol Rep ; 17(3): 14, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25740703

RESUMO

The association between antiphospholipid antibodies (aPL) and clinical problems goes beyond what is stated in the antiphospholipid syndrome (APS) classification criteria, namely thrombosis and pregnancy morbidity, and thrombocytopenia is the most common non-criteria hematologic manifestation of aPL with a frequency ranging from 20 to 50 %. Thrombocytopenia is rarely severe, and hemorrhage is far less common than thrombosis. However, when anticoagulation is considered, it may constitute a clinical problem with increased bleeding risk. Furthermore, thrombocytopenia represents a risk factor for thrombosis in aPL-positive patients. Therefore, it is important to understand the pathogenesis and the clinical associations of thrombocytopenia to build the right medical approach in aPL-positive patients. In this paper, we review the literature on aPL/APS-associated thrombocytopenia and briefly discuss the other conditions that can result in thrombocytopenia as they have commonalities with APS and their recognition is important to establish the most appropriate treatment strategy.


Assuntos
Síndrome Antifosfolipídica/complicações , Trombocitopenia/tratamento farmacológico , Trombocitopenia/etiologia , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Prednisona/uso terapêutico , Resultado do Tratamento
9.
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
10.
Thromb Res ; 129(4): 486-91, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22036125

RESUMO

BACKGROUND: Pathogenesis of thrombus formation in antiphospholipid syndrome (APS) is not clear. Platelet membrane glycoprotein (GP) receptors play important roles in development of thrombosis. OBJECTIVES: We investigated the association between development of thrombosis in APS and polymorphisms of GPIb alpha variable number of tandem repeats (VNTR), Kozak, and GPIa C807T. Patients/Methods Sixty patients with APS (30 with proven thrombosis and 30 without thrombosis) and 63 controls were included. Presence of GPIa C807T polymorphism was determined with real-time PCR and GPIb alpha Kozak and VNTR polymorphisms by conventional PCR. RESULTS: Frequency of C807T TT genotype was significantly higher in APS with thrombosis than APS without thrombosis (p=0.023) and also in APS with multiple thrombi compared to APS without thrombi (p=0.023). Frequency of Kozak TC genotype was higher in APS with arterial thrombosis compared to APS with venous thrombosis, controls, and APS without thrombosis (p=0.03, p=0.0007, and p=0.0024 respectively). D allele frequency and D allele carrier state for VNTR were significantly less in APS than controls (p=0.0018 and p=0.0046 respectively). CONCLUSIONS: C807T TT genotype may confer a risk for thrombosis and Kozak TC genotype for arterial thrombosis. D allele of VNTR may protect from APS. No patients with C807T TT or Kozak TC genotypes carried the protective DD genotype of VNTR. These polymorphisms may increase risk for both arterial and venous thrombosis. The utility of prophylaxis with anti-platelet drugs in at least a subgroup of APS patients should be investigated with clinical trials.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/genética , Predisposição Genética para Doença/epidemiologia , Predisposição Genética para Doença/genética , Complexo Glicoproteico GPIb-IX de Plaquetas/genética , Trombose/epidemiologia , Trombose/genética , Adulto , Comorbidade , Feminino , Humanos , Masculino , Polimorfismo de Nucleotídeo Único/genética , Prevalência , Medição de Risco , Fatores de Risco , Turquia/epidemiologia
11.
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
12.
J Thromb Thrombolysis ; 29(3): 303-9, 2010 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19504051

RESUMO

In this study, we evaluated common inherited thrombophilic risk factors in patients with antiphospholipid syndrome (APS), and reviewed relevant literature. Ninety-four APS patients with documented thrombosis, 40 patients with persistent antiphospholipid antibody (aPLA) positivity but without thrombosis, and healthy controls were screened. We found that inherited protein C, protein S, and antithrombin deficiencies were rare in APS patients. The presence of factor V Leiden G506A (FVL) mutation was significantly higher in APS patients with thrombosis compared to healthy controls (11.2% versus 4.9%, P = 0.0043). The prevalence of prothrombin G20210A mutation, however was not significantly increased in APS patients with thrombosis compared to patients without thrombosis (2.7% versus 1.25%, P = 0.67). Our literature review suggested that FVL mutation was associated with both arterial and venous thrombosis but prothrombin G20210A mutation does not seem to contribute much to thrombotic risk in patients with APS. In conclusion, the presence of FVL mutation may define a small but important subgroup of patients who had high risk of both venous and arterial thrombosis. Known thrombophilic risk factors however, may influence the development of thrombotic complications in approximately 10% of APS patients. These findings may indicate that thrombotic complications in APS patients are largely related with aPLA-mediated mechanisms.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/genética , Trombofilia/epidemiologia , Trombofilia/genética , Alanina/genética , Substituição de Aminoácidos/genética , Síndrome Antifosfolipídica/sangue , Fator V/genética , Glicina/genética , Humanos , Mutação/genética , Prevalência , Fatores de Risco , Trombofilia/sangue
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.
Amyloid ; 16(4): 226-31, 2009 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19922335

RESUMO

We report two patients who suffered from symmetrical polyarthritis simulating rheumatoid arthritis. Acute phase response was almost within normal limits, and autoantibodies including rheumatoid factor were negative. Both of them were diagnosed as having amyloid arthropathy (AmyA) secondary to kappa multiple myeloma based on deposition of kappa-light chain-immunoreactive amyloid in biopsied tissue and Bence Jones protein in urine. Systemic AL amyloidosis may be important in the differential diagnosis of chronic polyarthralgia.


Assuntos
Amiloidose/diagnóstico , Artrite Reumatoide/diagnóstico , Artropatias/diagnóstico , Artropatias/etiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/fisiopatologia , Idoso , Amiloidose/metabolismo , Amiloidose/urina , Artrite Reumatoide/etiologia , Artrite Reumatoide/patologia , Proteína de Bence Jones/urina , Feminino , Humanos , Pessoa de Meia-Idade , Mieloma Múltiplo/metabolismo
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