Nodular Scleritis in Association with Panuveitis in Behçet’s Disease

This case report involves a 32-year-old man with Behçet’s disease who had simultaneous bilateral anterior uveitis, unilateral nodular scleritis, and occlusive vasculitis with retinal hemorrhages. Although scleritis is not a classical feature of Behçet’s disease, a diagnosis of Behçet’s disease should be considered in patients with scleritis

Bilateral Intravitreal Ranibizumab Injection and Panretinal Photocoagulation in a 16-Year-Old Girl with Severe Vaso-Occlusive Lupus Retinopathy

A 16-year-old girl with fever of unknown origin and bilateral vaso-occlusive retinopathy with retinal neovascularization, preretinal hemorrhage, and serous macular detachment was treated with single bilateral 0.5 mg intravitreal ranibizumab injection prior to aggressive PRP with success. No systemic steroids or immunosuppressive therapy was employed at that time. She received the diagnosis of “systemic lupus erythematosus” five years after this episode with further systemic symptoms. In certain cases with vaso-occlusive type of lupus retinopathy, anti-VEGF agents may be administered in addition to panretinal photocoagulation to achieve better visual and anatomic outcome

Fetuin-A is related to syndesmophytes in patients with ankylosing spondylitis: a case control study

OBJECTIVES: New bone formation is one of the hallmark characteristics of ankylosing spondylitis, which is thereby associated with syndesmophytes. Fetuin-A is a molecule that is abundantly found in calcified tissues and it shows high affinity for calcium phosphate minerals and related compounds. Considering the role of fetuin-A in the regulation of calcified matrix metabolism, we compared the fetuin-A levels in ankylosing spondylitis patients with syndesmophytes with those in patients without syndesmophytes and in healthy controls. We also studied other biomarkers that are thought to be related to syndesmophytes. METHODS: Ninety-four patients (49 patients without syndesmophytes, 67.3% male, 40.7±8.7 years; 45 patients with syndesmophytes, 71.1% M, 43.9±9.9 years) and 68 healthy controls (44.2±10.6 years and 70.6% male) were included in this study. Syndesmophytes were assessed on the lateral radiographs of the cervical and lumbar spine. The serum levels of fetuin-A, dickkopf-1, sclerostin, IL-6, high-sensitivity C-reactive protein and bone morphogenetic protein-7 were measured with an enzyme-linked immunosorbent assay. RESULTS: Patients with syndesmophytes had significantly higher levels of fetuin-A compared with patients without syndesmophytes and controls (1.16±0.13, 1.05±0.09 and 1.08±0.13 mg/ml, respectively). However, fetuin-A was not different between the patients without syndesmophytes and controls. Bone morphogenetic protein-7 was significantly lower; dickkopf-1 was significantly higher in patients with ankylosing spondylitis compared with controls. The sclerostin concentrations were not different between the groups. In regression analysis, fetuin-A was an independent, significant predictor of syndesmophytes. CONCLUSION: Our results suggest that fetuin-A may a role in the pathogenesis of bony proliferation in ankylosing spondylitis

High prevalence of spondyloarthritis and ankylosing spondylitis among familial Mediterranean fever patients and their first-degree relatives: further evidence for the connection

INTRODUCTION: Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by recurrent attacks of fever and serositis. Limited data suggest that the prevalence of sacroiliitis is increased in patients with FMF. In our present study, we assessed the prevalence of spondyloarthritis (SpA), including ankylosing spondylitis (AS), among a cohort of FMF patients and their unaffected first-degree relatives (FDRs). METHODS: The current study cohort comprised a consecutive group of 201 unrelated patients with FMF and 319 FDRs (≥ 16 years old). These subjects were examined according to a standard protocol. RESULTS: A total of 157 FMF patients (78.1%) and 233 (73%) unaffected FDRs reported back pain. Fifteen FMF patients (7.5%) and nine unaffected FDRs fulfilled the modified New York (mNY) criteria for AS. One additional FDR with AS was identified after review of the medical records. None of the FMF patients with AS was HLA-B27 positive. The allele frequency of M694V among the FMF patients with radiographic sacroiliitis was significantly higher in comparison with those without sacroiliitis (OR 4.3). When compared with the general population, the risk ratios for SpA and AS among the FDRs of our FMF patients were 3.3 (95% CI; 2.0 to 5.5) and for AS 2.9 (95% CI; 1.3 to 6.4), respectively. CONCLUSIONS: Our study suggests that a) factors other than HLA-B27 play a role in the association of FMF and SpA/AS; b) MEFV gene variations may be one of the geographic/region-specific potential pathogenetic links between these two disorders in the Turkish population

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ABSTRACT. Objective. To estimate the prevalence of inflammatory back pain (IBP) and axial spondyloarthritis (axSpA) using the Assessment of SpondyloArthritis International Society (ASAS) classification criteria among employees in a university. Methods. In the first stage of the study, a face-to-face interview was done using a standard questionnaire to investigate IBP in 381 subjects randomly selected from 2894 employees at Dokuz Eylul University in Izmir, Turkey. In the second stage, subjects with back pain for ≥ 3 months and age at onset < 45 years were evaluated for axSpA using the ASAS criteria. Both the European Spondyloarthropathy Study Group (ESSG) criteria and Amor criteria were used for the classification of the whole group of spondyloarthritis (SpA). Results. There were 131 male and 250 female subjects (mean age: 38.0 yrs). Twenty-five subjects (6.6%) were classified as having IBP according to the ASAS criteria. The prevalence of IBP according to the Berlin and Calin criteria was 7.1% and 21.5%, respectively. The prevalence of axSpA was estimated at 1.3% according to the ASAS classification criteria (0.5% for radiographic axSpA and 0.8% for nonradiographic axSpA). A total of 7 patients (1.8%) fulfilled both the Amor and ESSG criteria for the whole group of SpA. Conclusion. This is the first prevalence study of IBP and axSpA using ASAS classification criteria in the Turkish population. Spondyloarthritides are among the most prevalent inflammatory rheumatic diseases 1 . There is a considerable diagnostic delay (8.9 yrs) in ankylosing spondylitis (AS), the prototype of this group, mainly because of the requirement of radiographic sacroiliitis for its diagnosis 2 . Low awareness of inflammatory back pain (IBP), the first and most common symptom of spondyloarthritis (SpA), in daily practice is also a major reason for the diagnostic delay 3 . New classification criteria developed by the Assessment of SpondyloArthritis International Society (ASAS) provide that patients with SpA can be classified as either patients with axial SpA (axSpA) or those with peripheral SpA. The ASAS axSpA criteria cover the entire spectrum of axial disease including AS and nonradiographic axSpA (nr-axSpA) MATERIALS AND METHODS We conducted our study at the Health Sciences Campus at Dokuz Eylul University in Izmir, which has 2894 medical and nonmedical staff aged between 18 and 67 years. A sample of 395 subjects was selected randomly by a computer from the list of all employees, based on the IBP prevalence of 5% in the general population 5 , using OpenEpi (version 2.3) and CI ± 2%. A total of 381 of these 395 subjects agreed to participate, an acceptance rate of 96.5%. In the first stage of the study, 6 trained medical students, using a standard questionnaire, interviewed participants face to face. Questionnaire responses were used to determine whether participants met the ASAS criteria for IBP 10 . Subjects were also evaluated for IBP based on the Berlin 11 and Calin criteria 12 In the second stage, the subjects with back pain for more than 3 month

A Case of Adult-Onset Still's Disease Complicated with Diffuse Alveolar Hemorrhage

Adult-onset Still's disease (AOSD) is an inflammatory disease that presents with a variety of clinical symptoms. Pulmonary involvement is well-known in AOSD and is seen in up to 53% of AOSD cases, with the most common pulmonary diseases being pleural effusion and transient pulmonary infiltrates. We present the first case of chronic AOSD complicated with diffuse alveolar hemorrhage during the acute flare of the disease

Analysis of the common genetic component of large-vessel vasculitides through a meta- Immunochip strategy

Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are major forms of large-vessel vasculitis (LVV) that share clinical features. To evaluate their genetic similarities, we analysed Immunochip genotyping data from 1,434 LVV patients and 3,814 unaffected controls. Genetic pleiotropy was also estimated. The HLA region harboured the main disease-specific associations. GCA was mostly associated with class II genes (HLA-DRB1/HLA-DQA1) whereas TAK was mostly associated with class I genes (HLA-B/MICA). Both the statistical significance and effect size of the HLA signals were considerably reduced in the cross-disease meta-analysis in comparison with the analysis of GCA and TAK separately. Consequently, no significant genetic correlation between these two diseases was observed when HLA variants were tested. Outside the HLA region, only one polymorphism located nearby the IL12B gene surpassed the study-wide significance threshold in the meta-analysis of the discovery datasets (rs755374, P?=?7.54E-07; ORGCA?=?1.19, ORTAK?=?1.50). This marker was confirmed as novel GCA risk factor using four additional cohorts (PGCA?=?5.52E-04, ORGCA?=?1.16). Taken together, our results provide evidence of strong genetic differences between GCA and TAK in the HLA. Outside this region, common susceptibility factors were suggested, especially within the IL12B locus