Budd-Chiari syndrome in Behcet's disease: a retrospective multicenter study

Objective To compare the clinical features, laboratory findings, and prognosis of Behget's disease (BD) patients with and without Budd-Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0 .005 and p = 0 .007) . Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0 .004) . Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls

Avaliação de entesopatia em pacientes com fibromialgia por meio do novo índice ultrassonográfico de entesite

OBJETIVO: Determinar a frequência de entesopatia na fibromialgia (FM) utilizando um método de ultrassonografia (US) recém-desenvolvido, o escore Madrid Sonography Enthesitis Index (MASEI). MÉTODOS: Este estudo foi realizado em 38 pacientes com FM consecutivos e 48 controles saudáveis pareados para idade e sexo. Seis sítios de ênteses (tuberosidade do olécrano, polos superior e inferior da patela, tuberosidade tibial, polos superior e inferior do calcâneo) nos dois membros inferiores foram avaliados. Todos os achados da US foram identificados de acordo com o escore MASEI. Os escores de pacientes e controles foram comparados usando-se o teste t de Student e o teste U de Mann-Whitney. A validade foi analisada pela curva receiver operating characteristic (ROC). Valores de P < 0,05 foram considerados significativos. RESULTADOS: O escore total de entesite foi 7,39 ± 4,99 (média ± DP) para os pacientes com FM e 3,7 ± 3,22 para os controles saudáveis (P < 0,001). A curva ROC estabeleceu um escore de US &gt; 3,5 no grupo de FM como o melhor ponto de corte para diferenciar casos de controles. Não houve correlação estatisticamente significativa entre o escore MASEI e a duração da FM, e a localização dos pontos dolorosos. CONCLUSÕES: Erros no diagnóstico de FM são prejudiciais aos pacientes e à comunidade, e a presença de entesopatia entre pacientes com FM é crescente. Sua detecção por meio do escore MASEI pode ser útil para discriminar pacientes com FM, cujos sintomas e sinais são mal definidos, para evitar equívoco de tratamento

Association Between The Presence Of Amyloidosis In Patients With Ankylosing Spondylitis and Polymorphisms In Type 1/Type 2 Serum Amyloid A Protein Genes, Mediterranean Fever Genes

77th Annual Meeting of the American-College-of-Rheumatology / 48th Annual Meeting of the Association-of-Rheumatology-Health-Professionals -- OCT 25-30, 2013 -- San Diego, CAWOS: 000325359202036…Amer Coll Rheumatol, Assoc Rheumatol Hlth Profes

The rate and significance of type 1/type 2 serum amyloid A protein gene polymorphisms in patients with ankylosing spondylitis and amyloidosis

WOS: 000361295200012PubMed ID: 26300108A relationship between the presence of amyloidosis and SAA1 genotype has been shown in recent studies of (principally) familial Mediterranean fever patients. We found that the SAA1 rs12218 polymorphism was significantly more prevalent in ankylosing spondylitis patients with amyloidosis

Thiol/disulfide homeostasis in patients with ankylosing spondylitis

Ankylosing spondylitis (AS) is a chronic inflammatory disease. In many inflammatory diseases, increased production of pro-inflammatory cytokines is associated with an increase in oxidative stress mediators. Thiol/disulfide homeostasis is a marker for oxidative stress. The aim of this study was to examine the dynamic thiol/disulfide homeostasis in AS. Sixty-nine patients with AS and 60 age- and sex-matched controls were included in the study. The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and visual analogue scale (VAS) were used to determine the disease activity. Native thiol, total thiol, and disulfide levels were measured with a novel automated method recently described by Erel and Neselioglu. The aforementioned method is also optionally manual spectrophotometric assay. The total thiol levels were significantly lower in the AS group compared with the control group (p = 0.03). When the patients were divided into active (n = 35) and inactive (n = 34) subgroups using BASDAI scores, the native plasma thiol and total thiol levels were significantly lower in the active AS patients compared to the inactive AS patients (p = 0.02, p = 0.03 respectively). There was a negative correlation between the plasma native thiol levels and VAS, BASDAI scores. Thiol/disulfide homeostasis may be used for elucidating the effects of oxidative stress in AS. Understanding the role of thiol/disulfide homeostasis in AS might provide new therapeutic intervention strategies for patients