Objective. To investigate the effect of infliximab on the frequency of uveitis attacks and the visual prognosis in male patients with Behçet's disease in whom uveitis was resistant to combination therapy with corticosteroids, azathioprine, and cyclosporine.Methods. The study group comprised patients who were receiving combination therapy but experienced at least 2 attacks of posterior uveitis/panuveitis or retinal vasculitis during the 6-month period prior to enrollment. Infliximab infusions (5 mg/kg) were administered at weeks 0, 2, 6, and 14. Weeks 0-22 were defined as the infusion period, and weeks 23-54 were defined as the observation period. Patients continued to receive azathioprine and corticosteroids, but cyclosporine was discontinued after the screening visit. The primary outcome measures were the absence of uveitis attacks during the infusion period (remission), and the absence of uveitis attacks throughout the study period (sustained remission).Results. Thirteen patients were enrolled in the study. Thirty-two uveitis attacks involving the posterior segment occurred during the previous-treatment period. During the infusion period, 4 patients (30.8%) remained attack-free, and 9 patients had a total of 13 uveitis attacks. Ten of these attacks (76.9%) occurred at either week 14 or week 22. One of 13 patients fulfilled the definition of sustained remission, and the remaining 12 patients had a total of 36 uveitis attacks during the observation period. The mean number of uveitis attacks and daily corticosteroid doses were significantly lower during the infusion period than during the previoustreatment period or the observation period. Although potential visual acuity was regained following infliximab infusion, this beneficial effect was not preserved until week 54. None of the patients experienced a serious adverse event.Conclusion. The results of this trial suggest that infliximab is effective in suppressing the occurrence of uveitis attacks, has a corticosteroid-sparing effect, and has favorable implications for the visual prognosis of patients with resistant Behçet's uveitis.Behçet's disease (BD) is a multisystem inflammatory disorder with a chronic, relapsing course. Patients with ocular involvement typically have bilateral nongranulomatous panuveitis and retinal vasculitis that involves all elements of the retinal vasculature (1). The course of the disease is characterized by recurrent, explosive attacks of uveitis followed by spontaneous remissions. The severity and frequency of these inflammatory episodes determine the extent of permanent damage to the intraocular structures and resultant visual loss. Permanent sequelae of posterior segment involvement include optic atrophy, maculopathy, sheathing and occlusion of retinal vessels, and diffuse atrophy and gliosis of the retina. Although the course of the disease shows individual variability, male patients have a younger age at disease onset, more severe disease, and
Takayasu arteritis is a rare inflammatory disease of large arteries. The etiology of Takayasu arteritis remains poorly understood, but genetic contribution to the disease pathogenesis is supported by the genetic association with HLA-B*52. We genotyped ~200,000 genetic variants in two ethnically divergent Takayasu arteritis cohorts from Turkey and North America by using a custom-designed genotyping platform (Immunochip). Additional genetic variants and the classical HLA alleles were imputed and analyzed. We identified and confirmed two independent susceptibility loci within the HLA region (r(2) < 0.2): HLA-B/MICA (rs12524487, OR = 3.29, p = 5.57 × 10(-16)) and HLA-DQB1/HLA-DRB1 (rs113452171, OR = 2.34, p = 3.74 × 10(-9); and rs189754752, OR = 2.47, p = 4.22 × 10(-9)). In addition, we identified and confirmed a genetic association between Takayasu arteritis and the FCGR2A/FCGR3A locus on chromosome 1 (rs10919543, OR = 1.81, p = 5.89 × 10(-12)). The risk allele in this locus results in increased mRNA expression of FCGR2A. We also established the genetic association between IL12B and Takayasu arteritis (rs56167332, OR = 1.54, p = 2.18 × 10(-8)).
It has been generally accepted that the clinical onset of familial Mediterranean fever (FMF) begins before 20 years of age in most patients. In this study, we aimed to investigate the demographic and clinical characteristics of our FMF patients with an age of onset > or =20. Records of 401 patients (female/male: 204/197) that followed up between 1990 and 1999 were reviewed according to a pre-defined protocol. All patients fulfilled the diagnostic criteria of Livneh et al. The demographic and clinical features of adult-onset FMF patients were compared to those of patients with a disease onset before 20 years of age. There were 57 patients (14%) who experienced symptoms of FMF at > or =20 years of age; 34 of them (8.5%) reported their first attack between 20 and 29 years of age; 18 of them (4.5%) between 30 and 39 years of age and five patients (1.25%) had their first attack after 40 years of age. Arthritis (42 vs. 65%, p = 0.001) and erysipelas-like erythema (7 vs. 17%, p = 0.047) were significantly less frequent in patients with adult-onset FMF compared to patients with disease onset before 20 years of age. Arthritis and erysipelas-like erythema were less frequent in adult-onset patients compared to those with an earlier disease onset. Adult-onset FMF may be a form of disease with distinct clinical, demographic and molecular characteristics. Prospective clinical studies and investigation of genotypic features are needed to identify the characteristics of this phenotypic variant.
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.
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