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1.
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.

2.
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
3.
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
4.
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.

5.
Rheumatol Int ; 39(2): 169-185, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30221327

RESUMO

There are no universally accepted diagnostic criteria for large-vessel vasculitides (LVV), including giant cell arteritis (GCA) and Takayasu arteritis (TAK). Currently, available classification criteria cannot be used for the diagnosis of GCA and TAK. Early diagnosis of these two diseases is quite challenging in clinical practice and may be accomplished only by combining the patient symptoms, physical examination findings, blood test results, imaging findings, and biopsy results, if available. Awareness of red flags which lead the clinician to investigate TAK in a young patient with persistent systemic inflammation is helpful for the early diagnosis. It should be noted that clinical presentation may be highly variable in a subgroup of GCA patients with predominant large-vessel involvement (LVI) and without prominent cranial symptoms. Imaging modalities are especially helpful for the diagnosis of this subgroup. Differential diagnosis between older patients with TAK and this subgroup of GCA patients presenting with LVI may be difficult. Various pathologies may mimic LVV either by causing systemic inflammation and constitutional symptoms, or by causing lumen narrowing with or without aneurysm formation in the aorta and its branches. Differential diagnosis of aortitis is crucial. Infectious aortitis including mycotic aneurysms due to septicemia or endocarditis, as well as causes such as syphilis and mycobacterial infections should always be excluded. On the other hand, the presence of non-infectious aortitis is not unique for TAK and GCA. It should be noted that aortitis, other large-vessel involvement or both, may occasionally be seen in various other autoimmune pathologies including ANCA-positive vasculitides, Behçet's disease, ankylosing spondylitis, sarcoidosis, and Sjögren's syndrome. Besides, aortitis may be idiopathic and isolated. Atherosclerosis should always be considered in the differential diagnosis of LVV. Other pathologies which may mimic LVV include, but not limited to, congenital causes of aortic coarctation and middle aortic syndrome, immunoglobulin G4-related disease, and hereditary disorders of connective tissue such as Marfan syndrome and Ehler-Danlos syndrome.


Assuntos
Arterite de Células Gigantes/diagnóstico , Arterite de Takayasu/diagnóstico , Diagnóstico Diferencial , Diagnóstico Precoce , Humanos
6.
Eur J Rheumatol ; 5(4): 249-253, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30501851

RESUMO

OBJECTIVE: Pulmonary hypertension (PH) may occur in Takayasu arteritis (TA), mostly due to pulmonary arteritis, but also due to left heart disease and/or chronic thromboembolism (CTE). Using transthoracic echocardiography (TTE), we investigated the frequency of PH caused by pulmonary arteritis. METHODS: This cross-sectional study included 70 patients with TA fulfilling the 1990 ACR criteria, 68 healthy controls, and 67 patients with systemic sclerosis (SSc) fulfilling the 1980 ACR criteria representing the disease control group. Patients with severe left heart disease or CTE were excluded. The ESC-ERS guideline definition was considered for diagnosis of PH. RESULTS: The mean systolic pulmonary artery pressure (SPAP) values in TA, SSc, and healthy control groups were 20.93±6.06, 31.57±12.75, and 18.88±5.39 mmHg, respectively. While the SPAP values were similar between TA and healthy groups, the SPAP values in the SSc group were significantly higher than in other groups. Based on conventional and/or magnetic resonance angiography findings, pulmonary arteritis was present in 4 out of 70 TA patients; however, PH was not detected in any patients with TA, including those with pulmonary arteritis. CONCLUSION: The TTE findings suggested that the frequency of PH was not increased in TA. However, a low frequency of pulmonary arteritis in our series might have affected our results.

7.
Mediterr J Hematol Infect Dis ; 10(1): e2018047, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30210740

RESUMO

Hemophagocytic Lymphohistiocytosis (HLH) is an indicator of an exaggerated immune response and eventually adverse outcomes. This study aimed to investigate the clinical and laboratory features and outcomes of patients with HLH. The medical records of 26 HLH adult patients (≥ 16 years of age) were retrospectively analyzed. Gender, age, the duration of fever, time to diagnosis, etiology and laboratory data were extracted from the records. The mean age was 38 ± 18 years, and 15 (58%) patients were female. A total of nine cases had infectious diseases; four cases had rheumatologic diseases, three cases had hematological malignancies while nine cases could not have a definitive diagnosis. The median time to detection of HLH was 20 days (IQR: 8-30 d). Of the 25 patients, 11 (44%) died. The erythrocyte sedimentation rates of the surviving and non-surviving patients were 39 ± 22 mm/h and 15 ± 13 mm/h, respectively. When a long-lasting fever is complicated by bicytopenia or pancytopenia (especially), clinicians should promptly consider the possibility of HLH syndrome to improve patients' prognosis.

8.
Turk J Med Sci ; 48(4): 681-697, 2018 Aug 16.
Artigo em Inglês | MEDLINE | ID: mdl-30114347

RESUMO

Takayasu arteritis (TAK) is a challenging chronic, granulomatous, large-vessel systemic vasculitis, mostly due to difficulties in early diagnosis and assessing actual disease activity. Since there are no specific diagnostic laboratory tests, biomarkers, or autoantibodies, many patients experience considerable delay in diagnosis. Remembering the possibility of TAK together with the use of acute phase responses and appropriate imaging studies may be helpful for early diagnosis. Since there may be discrepancies between systemic and vascular wall inflammation, using only acute phase responses is not reliable in assessing current disease activity. Therefore, physical examination and new imaging findings should also be used to assess current disease activity. Despite its limitations, the Indian Takayasu Clinical Activity Score (ITAS2010) may also be helpful for this purpose. The rationale of medical treatment is to suppress both vascular and systemic inflammation with appropriate systemic immunosuppression, including corticosteroids and conventional immunosuppressive agents. In cases of refractory disease activity, leflunomide and biologic agents such as TNF inhibitors and tocilizumab may be tried. In selected cases with persistent lesions that cannot be reversed with medical treatment, endovascular interventions including balloon angioplasty, stent and stent graft replacement, or surgery may be tried. However, such procedures should be performed after suppression of inflammation, i.e. during inactive disease. Prognosis of TAK is probably getting better with lower mortality rates reported in recent years, probably due to the use of more effective medical treatments as well as the use of endovascular interventions when necessary and available.


Assuntos
Procedimentos Endovasculares/métodos , Imunossupressores/uso terapêutico , Arterite de Takayasu , Anticorpos Monoclonais Humanizados/uso terapêutico , Biomarcadores/metabolismo , Guias como Assunto , Humanos , Leflunomida/uso terapêutico , Exame Físico , Prognóstico , Radiologia Intervencionista , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/fisiopatologia , Arterite de Takayasu/terapia
9.
Int J Rheum Dis ; 21(4): 880-884, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29314707

RESUMO

AIM: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. One of the common characteristics of this disease is its young age predominance. Nearly 90% of patients experience disease flares during early adult age periods. Currently there are limited data for the comparison of early versus late onset FMF and therefore the primary aim of this study was to investigate these two subsets with regard to their certain demographic, clinical and genetic differences. METHODS: Early (≤ 20 years, Group 1) and late (> 20 years, Group 2) onset FMF patients were identified from the national FMF registry that involves 2246 patients from 15 adult rheumatology clinics located in different geographical areas of Turkey. RESULTS: Of the 2246 patients, 1633 (72.7%) were aged ≤ 20 years old (Group 1) and the remaining 613 were older than 20 years (Group 2). Delay in diagnosis was longer in Group 1 and fever, peritonitis, pleuritis, erysipelas-like erythema (ELE), arthritis, family history of FMF and amyloidosis were more common in Group 1. On the other hand, sex distribution, rates of amyloidosis, vasculitis and kidney failure were not different between the groups. Among patients with available genotypes, homozygous and heterozygous M694V mutations were significantly higher and heterozygous E148Q mutation was significantly lower in Group 1 compared to Group 2. CONCLUSION: Patients with FMF whose symptoms start before 20 years of age seem to have severe symptoms and M694V mutation may be responsible for the early expression of the disease.


Assuntos
Febre Familiar do Mediterrâneo/genética , Mutação , Pirina/genética , Adolescente , Adulto , Idade de Início , Criança , Diagnóstico Tardio , Progressão da Doença , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/epidemiologia , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Taxa de Mutação , Fenótipo , Valor Preditivo dos Testes , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Distribuição por Sexo , Fatores de Tempo , Turquia/epidemiologia , Adulto Jovem
10.
Arthritis Care Res (Hoboken) ; 70(7): 1090-1094, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-28992387

RESUMO

OBJECTIVE: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. RESULTS: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. CONCLUSION: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.


Assuntos
Sistemas de Liberação de Medicamentos/métodos , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/epidemiologia , Interleucina-1/administração & dosagem , Uso Off-Label , Adolescente , Adulto , Idoso , Febre Familiar do Mediterrâneo/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Turquia/epidemiologia , Adulto Jovem
11.
Rheumatology (Oxford) ; 57(5): 784-790, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-28968895

RESUMO

A lack of absolute correlation between systemic inflammation parameters and ongoing vascular disease activity is an important problem in some patients with large vessel vasculitis, especially Takayasu arteritis (TAK). Systemic and vascular wall inflammation in TAK are obviously interrelated, but sometimes they may act independently. There are clear discrepancies between these two types of inflammation, including cytokine patterns and responses to treatment. Vascular and systemic inflammation may also be discordant in two subgroups of giant cell arteritis. The first subgroup is mainly characterized by severe systemic inflammation mostly associated with IL-6-driven immunity, while in the second subgroup there is less systemic inflammation but prominent neuro-ophthalmic ischaemic complications characterized mostly by IFN-γ-mediated effects. Although no definite boundaries exist, it may be suggested that the IL-6/Th17/IL-17 pathway primarily drives systemic inflammation while the IL-12/Th1/IFN-γ pathway dominates in vascular wall inflammation both in TAK and giant cell arteritis. Immunosuppressive treatment of TAK (especially corticosteroids) initially suppresses systemic inflammation, while longer treatment duration is required for the suppression of vascular inflammation. Therefore, evaluating only the systemic inflammation may be misleading. Vascular wall inflammation is currently evaluated using expensive imaging methods, which are not feasible for repetitive use. Although pentraxin-3 is superior to erythrocyte sedimentation rate and CRP, we need more reliable biomarkers to reflect vascular wall inflammation in patients with TAK.


Assuntos
Vasos Sanguíneos/patologia , Proteína C-Reativa/metabolismo , Citocinas/sangue , Arterite de Células Gigantes/diagnóstico , Inflamação/diagnóstico , Componente Amiloide P Sérico/metabolismo , Arterite de Takayasu/diagnóstico , Biomarcadores/metabolismo , Vasos Sanguíneos/metabolismo , Diagnóstico Diferencial , Arterite de Células Gigantes/metabolismo , Humanos , Inflamação/metabolismo , Arterite de Takayasu/metabolismo
12.
Presse Med ; 46(7-8 Pt 2): e229-e235, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28774475

RESUMO

Management of Takayasu arteritis (TAK) is challenging mostly due to difficulties in assessing actual disease activity. The rational of medical treatment is to suppress both vascular and systemic inflammation with appropriate systemic immunosuppression, including corticosteroids and conventional immunosuppressive (IS) agents. In case of refractory disease activity, biologic agents such as TNF inhibitors and tocilizumab may be tried. In selected cases, endovascular interventions and surgical procedures may be indicated and should be performed during inactive disease. Among conventional IS agents, new data is available for leflunomide. On the other hand, most of the new information in the management of TAK arises from the growing experience with biologic agents used in resistant cases. Besides, there are potential new therapeutic targets which may be promising in the future for medical treatment of TAK. Finally, new trends in endovascular interventions for management of TAK deserve attention.


Assuntos
Arterite de Takayasu/terapia , Abatacepte/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Procedimentos Endovasculares , Humanos , Fatores Imunológicos/uso terapêutico , Imunossupressores/uso terapêutico , Radiologia Intervencionista , Rituximab/uso terapêutico
13.
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
14.
Rheumatol Int ; 36(7): 945-53, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27221456

RESUMO

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy-based on creatinine level, proteinuria and disease activity-was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.


Assuntos
Amiloidose/tratamento farmacológico , Artrite Reumatoide/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Imunossupressores/uso terapêutico , Espondilite Anquilosante/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Idoso , Amiloidose/diagnóstico , Amiloidose/epidemiologia , Amiloidose/imunologia , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/imunologia , Produtos Biológicos/efeitos adversos , Progressão da Doença , Feminino , Humanos , Hospedeiro Imunocomprometido , Imunossupressores/efeitos adversos , Incidência , Masculino , Pessoa de Meia-Idade , Infecções Oportunistas/induzido quimicamente , Infecções Oportunistas/epidemiologia , Infecções Oportunistas/imunologia , Prevalência , Indução de Remissão , Estudos Retrospectivos , Fatores de Risco , Espondilite Anquilosante/diagnóstico , Espondilite Anquilosante/epidemiologia , Espondilite Anquilosante/imunologia , Fatores de Tempo , Resultado do Tratamento , Tuberculose/induzido quimicamente , Tuberculose/epidemiologia , Tuberculose/imunologia , Fator de Necrose Tumoral alfa/imunologia , Turquia/epidemiologia
15.
J Autoimmun ; 69: 59-63, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26970681

RESUMO

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.


Assuntos
Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios/uso terapêutico , Biomarcadores , Diagnóstico Tardio , Quimioterapia Combinada , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Recidiva , Indução de Remissão , Fatores de Risco , Resultado do Tratamento , Adulto Jovem
16.
J Rheumatol ; 43(5): 893-900, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-26834222

RESUMO

OBJECTIVE: To assess the performance of the new 2012 provisional European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) polymyalgia rheumatica (PMR) clinical classification criteria in discriminating PMR from other mimicking conditions compared with the previous 5 diagnostic criteria in a multicenter prospective study. METHODS: Patients older than 50 years, presenting with new-onset bilateral shoulder pain with elevated acute-phase reactants (APR), were assessed for the fulfillment of the new and old classification/diagnostic criteria sets for PMR. At the end of the 1-year followup, 133 patients were diagnosed with PMR (expert opinion) and 142 with non-PMR conditions [69 rheumatoid arthritis (RA)]. Discriminating capacity, sensitivity, and specificity of the criteria sets were estimated. RESULTS: Discriminating capacity of the new clinical criteria for PMR from non-PMR conditions and RA as estimated by area under the curve (AUC) were good with AUC of 0.736 and 0.781, respectively. The new criteria had a sensitivity of 89.5% and a specificity of 57.7% when tested against all non-PMR cases. When tested against all RA, seropositive RA, seronegative RA, and non-RA control patients, specificity changed to 66.7%, 100%, 20.7%, and 49.3%, respectively. Except for the Bird criteria, the 4 previous criteria had lower sensitivity and higher specificity (ranging from 83%-93%) compared with the new clinical criteria in discriminating PMR from all other controls. CONCLUSION: The new 2012 EULAR/ACR clinical classification criteria for PMR is highly sensitive; however, its ability to discriminate PMR from other inflammatory/noninflammatory shoulder conditions, especially from seronegative RA, is not adequate. Imaging and other modifications such as cutoff values for APR might increase the specificity of the criteria.


Assuntos
Artrite Reumatoide/diagnóstico , Polimialgia Reumática/diagnóstico , Dor de Ombro/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polimialgia Reumática/classificação , Estudos Prospectivos , Sensibilidade e Especificidade
17.
Clin Exp Rheumatol ; 34(3 Suppl 97): S73-6, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26885720

RESUMO

OBJECTIVES: To date, no biomarker is universally accepted to be a surrogate for active disease being one of major difficulties in follow-up of Takayasu's arteritis (TAK). In this study, we aimed to investigate plasma pentraxin-3 (PTX-3) levels and its correlation with activity in patients with TAK. METHODS: This cross-sectional study included 94 patients (age: 43.3±13.6 years, F/M: 80/14) with TAK, 40 age-sex matched control donors (age: 41.5±9.3 years, F/M: 28/12). TAK patients were evaluated by physician's global assessment (PGA; active/inactive), as well as with the activity definition by Kerr et al. and with a new composite index of ITAS2010 (Indian Takayasu Clinical Activity Score). Plasma PTX-3 levels are measured with an enzyme linked immunosorbent assay kit. RESULTS: Thirty-three (35.5%) patients were clinically active with PGA, while 25 (31.6%) patients and 28 (31.8%) patients were accepted to have active disease according to Kerr activity criteria and ITAS2010, respectively. Plasma PTX-3 levels were significantly higher in TAK patients compared to healthy controls (3.5±2.5 ng/ml vs. 2.5±1.6 ng/ ml, p=0.029). However, PTX-3 levels were similar among active and inactive patients according to all three assessment tools. PTX-3 levels significantly correlated only with serum CRP levels. CONCLUSIONS: Although plasma PTX- 3 levels were higher in patients with TAK compared to healthy controls, we observed no association with disease activity, limiting the role of PTX-3 level as a biomarker for active disease in TAK.


Assuntos
Proteína C-Reativa/análise , Componente Amiloide P Sérico/análise , Arterite de Takayasu/sangue , Adulto , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade
19.
Wien Klin Wochenschr ; 128(21-22): 809-815, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25860849

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), in which pulmonary involvement often predominates, is a multisystem granulomatous, necrotizing vasculitis that affects small and medium-sized vessels. In this study we evaluated various radiological findings of pulmonary GPA and focused on spiculated pulmonary lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy. METHODS: This retrospective study included 48 patients, aged 28-73 (mean, 47.3) years, who showed either histopathological diagnosis of GPA (n = 39) or elevated levels of the cytoplasmic anti-neutrophilic cytoplasmic antibody serum marker (n = 9) between January 2003 and December 2013. All patients received a chest computed tomography (CT), and the types of pulmonary lesions were defined and evaluated. RESULTS: Among the 48 patients, 33 had abnormal pulmonary findings on CT. The most commonly detected pulmonary lesion types were nodules and masses (n = 126) observed in 24 patients. Cavitation, necrosis, spiculation and invasion of the fissure, pleura or diaphragm were observed in 14, 9, 10 and 6 patients, respectively. Consolidation was found in 14 patients and thickening of bronchial wall in 8 patients. CONCLUSIONS: Pulmonary lesion types of GPA have a wide spectrum, potentially mimicking a high number of diseases including malignancy, infection and noninfectious inflammatory diseases. A spiculated lung lesion invading the fissure, pleura or diaphragm is mostly present in malignancy, but it can be also seen in GPA.


Assuntos
Diafragma/diagnóstico por imagem , Granulomatose com Poliangiite/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Pleura/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Diagnóstico Diferencial , Granulomatose com Poliangiite/complicações , Humanos , Pneumopatias/complicações , Neoplasias Pulmonares/diagnóstico por imagem , Pessoa de Meia-Idade , Radiografia Torácica/métodos , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
20.
Clin Exp Rheumatol ; 34(3 Suppl 97): S33-9, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26633698

RESUMO

OBJECTIVES: To estimate the prevalence and incidence of Takayasu arteritis (TA) among the residents of the city of Izmir, the third largest metropolis in Turkey. METHODS: Five tertiary care teaching hospitals, which were the only ones that provided rheumatology specialty care during the study period in the city of Izmir from 2006 through 2010, were invited to take part in the present study. A case search was performed electronically in the information systems of these hospitals using The International Classification of Diseases Tenth Revision (ICD-10) code for Takayasu arteritis (M31.4). The diagnosis was confirmed through chart review by a rheumatologist according to the 1990 American College of Rheumatology (ACR) criteria. Annual prevalence was calculated based on the number of patients that were alive at the end of 2010. Age- and sex-adjusted prevalence rates were standardised according to the 2010 Turkish population, based on 2010 Turkish Census. RESULTS: A total of 41 patients were confirmed to have TA and also to live within the targeted area. The annual prevalence was estimated as 12.8 (95% CI 12.0-13.6) per million; 23.5/million (95% CI 21.9-25.0) in females and 1.9/million (95% CI 1.5-2.4) in males. The prevalence was higher 8.8/million (95% CI 7.7-10.0) in the population >40 years of age. During the study period, the mean annual incidence of TA was estimated as 1.11/million (95% CI 0.54-1.67). CONCLUSIONS: The first epidemiologic study of TA in a Turkish population suggests that TA is a relatively common vasculitis in Turkey.


Assuntos
Arterite de Takayasu/epidemiologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Turquia/epidemiologia
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