European journal of rheumatology最新文献

Background: Behçet's disease is a systemic vasculitis affecting both arteries and veins, as well as caus- ing recurrent inflammatory multiorgan disease. Vascular involvement is associated with increased mortality and morbidity. Matrix metalloproteinases are released at sites of inflammation and degrade various components of the extracellular matrix. Increased levels of metalloproteinase-9 and metal- loproteinase-2 have been previously reported in Behçet's disease.

Methods: In this cross-sectional study, metalloproteinase-2 and metalloproteinase-3 serum levels were investigated in 103 patients with Behçet's disease and 69 healthy controls, using Invitrogen immunoassay human metalloproteinase-2 and metalloproteinase-3 ELISA kits.

Results: Serum metalloproteinase-2 and metalloproteinase-3 levels were significantly higher in the Behçet's disease group compared to healthy controls. Besides, serum metalloproteinase-3 levels were significantly higher in subgroups of Behçet's disease with aneurysmal vascular involvement and with neurological involvement. However, metalloproteinase-2 and metalloproteinase-3 serum levels did not show a positive correlation with disease activity.

Conclusion: Metalloproteinase-2 and -3 may contribute to the complex pathogenesis of Behçet's dis- ease. More importantly, the detection of very high serum levels of metalloproteinase-3 may predict the formation of an aneurysm, or possibly the presence of neurological involvement in Behçet's dis- ease and may lead the clinician to make an earlier diagnosis of these complications in young male patients with high risk.

Background: To investigate whether sarcopenic obesity may contribute to knee osteoarthritis or not.

Methods: In this study, we assessed 140 community-dwelling adult patients. Their demographic data were recorded along with comorbidities. Anterior mid-thigh muscle thickness in the axial plane was measured on the dominant leg using ultrasound midway between the anterior superior iliac spine and the upper end of patella in millimeter. Then, the sonographic thigh adjustment ratio was calcu- lated by dividing this thickness by body mass index. ISarcoPRM algorithm was used for the diagnosis of sarcopenia. Kellgren-Lawrence grading was used for knee osteoarthritis . Functional evaluation was performed using chair stand test, gait speed, and grip strength.

Results: There were 50 patients with knee osteoarthritis and 90 age- and gender-similar control sub- jects. When compared with controls, anterior thigh muscle thickness, gait speed, and grip strength were found to be similar between the groups, whereas body mass index and chair stand test val- ues were higher in the knee osteoarthritis group (both P < .05). In addition, sarcopenic obesity was observed in 12 (13.3%) of control subjects and in 14 (28%) of osteoarthritis patients. When age, gen- der, exercise, smoking, and body composition type (i.e., nonsarcopenic nonobese, sarcopenic only, obese only, and sarcopenic obesity) were taken into binary logistic regression analyses, only sarcope- nic obesity [relative risk ratio = 2.705 (95% CI: 1.079-6.779)] was independently related with the knee osteoarthritis (P < .05).

Conclusion: Our preliminary study has shown that neither sarcopenia nor obesity but sarcopenic obe- sity seems to be independently related to the knee osteoarthritis. Further longitudinal studies with larger samples are required for investigating the effects of obesity and sarcopenia on the develop- ment of knee osteoarthritis.

The ocular inflammatory process may be associated with autoimmune inflammatory joint damage and can be better recovered by B-mode ultrasound, being little explored in the absent eye evaluation. This study aimed to conduct a systematic review using the Patients or Problem, Intervention, Control or Comparison, Outcomes strategy: uveitis; ultrasound, arthritis, and diagnosis. Clinical trials, meta-analysis, and randomized controlled trials that specifically address the scope of this study will be evaluated. For the search in the database, a choice of controlled vocabulary will be used with the MEDLINE MeSH platform (Medical Subject Headings). The articles must be dated from the year 2010 until the year 2020. To charting methods will be used Preferred Reporting Items for Systematic Reviews and Meta-Analyses Flow Diagram and risk of bias: the Cochrane risk of bias tool. Grade of recommendation assessment: Grading of Recommendations Assessment, Development, and Evaluation Group guidelines. Of 2909 studies, only 13 studies were included, which evaluated the use of B-mode ultrasound to assess anterior and intermediate uveitis and complications, and 5 cases showed an association of vitreitis. B-mode ultrasound can be an important benefit of complementing clinical evaluation in patients with the uveal inflammatory process associated with several autoimmune arthropathies, but more studies with better-elaborated methodology design will be necessary.

Objective: Vedolizumab is a novel anti-inflammatory molecule that is currently being used in the treatment of refractory inflammatory bowel disease. The mode of action is inhibiting the binding of activated T lymphocytes to the adhesion molecule 1 of intestinal mucosal cells. Due to its local effect, systemic immunosuppression is not expected, and this may have a negative effect on the extra-intestinal symptoms of inflammatory bowel disease, particularly spondyloarthritis. Currently, there is limited data regarding the effect of vedolizumab on spondyloarthritis symptoms. We aimed to investigate whether vedolizumab has an effect on the occurrence of rheumatological symptoms and the clinical course of patients who have spondyloarthritis.

Methods: Thirty-nine adult inflammatory bowel disease patients who were followed up in the Gastroenterology Clinic and treated with vedolizumab were included in the study. Patients were reviewed in terms of rheumatological manifestations. The occurrence of new musculoskeletal findings during the vedolizumab treatment was recorded. Patients with a former diagnosis of spondyloarthritis were evaluated for the activity of axial and peripheral manifestations during the vedolizumab.

Results: There were 39 inflammatory bowel disease patients (29 Crohn's disease, 10 ulcerative colitis, 48.7% (n = 19) male) who had been treated with vedolizumab. The mean age of the patients was 41.4 ± 15.7 years, and the duration of inflammatory bowel disease was 10.4 ± 7.5 years. A total of 17 (44%) patients had accompanying spondyloarthritis findings (mean age 47.08 ± 15.325 years and 58.8% M). Seven patients had axial dominant symptoms and 6 of them were in an active disease state before vedolizumab. During vedolizumab, all but 1 continued to be active. There were 14 patients with arthritis/arthralgias before vedolizumab and only 3 had improvement with therapy. On the other hand, there were 3 patients who had new-onset arthralgias/arthritis with vedolizumab. In total, 6 patients needed to stop vedolizumab because of spondyloarthritis activation (n = 2) and uncontrolled inflammatory bowel disease (n = 4), respectively.

Conclusion: Treatment with vedolizumab seems no effect on both the occurrence and the course of rheumatological manifestations in inflammatory bowel disease patients. Further studies are required to replicate our results.

Rosai-Dorfman disease is characterized by dilated lymph node sinuses filled with lymphocytes, plasma cells, and histiocytes. Many of these histiocytes classically exhibit emperipolesis of lymphocytes and plasma cells. Abundant immunoglobulin G4+ plasma cells occur in some cases, and a potential relationship with immunoglobulin G4-related disease has been suggested. Here, we report 3 cases of immunoglobulin G4-associated Rosai-Dorfman disease. Immunoglobulin G4-related disease was suspected based on immunoglobulin G4+ plasma cell infiltration, but the final diagnosis was immunoglobulin G4-associated Rosai-Dorfman disease. At present, the evidence does not support a link between immunoglobulin G4-associated Rosai-Dorfman disease and immunoglobulin G4-related disease, and one condition should not be considered part of the spectrum of the other. We believe it is of paramount importance to increase the awareness of immunoglobulin G4-associated Rosai-Dorfman disease for pathologists who interpret the biopsies and clinicians who integrate the diagnosis and treat such patients to not overdiagnose immunoglobulin G4-related disease.

Behcet's syndrome is a variable vessel vasculitis characterized by recurrent oral and genital ulcers with concomitant skin, ocular, neurologic, gastrointestinal, and joint involvement. Herein, we present a patient who was diagnosed with Behcet's syndrome, which with magnetic resonance angiography showed occlusion of the right subclavian artery at the level of the thoracic outlet and reverse flow in the right vertebral artery consistent with subclavian steal syndrome. In addition, partial narrowing was noted in the left renal artery. The distribution of arterial involvement resembled Takayasu's arteritis, although the presence of mucocutaneous lesions, male gender, history of deep vein thrombosis, and HLA-B51 positivity favored a diagnosis of vasculo-Behçet's syndrome. We treated the patient with methylprednisolone and cyclophosphamide. After the regression of vascular inflammation with immunosuppressive therapy, stenting was performed in the left renal artery.

Hypereosinophilic syndrome requires a peripheral absolute eosinophil count of ≥1.5 × 109 /L with clinical manifestations attributable to peripheral or tissue hypereosinophilia. Clinical manifestations can vary greatly, with the majority of patients relatively asymptomatic and the eosinophilia detected incidentally. However, in a minority of hypereosinophilia cases, they may present with severe lifethreatening organ dysfunction affecting skin, lung, heart, gastrointestinal tract, and nervous system. A case of hypereosinophilia with potentially life-threatening cardiovascular involvement is discussed. Initial laboratory investigations showed an elevated white blood cell count with 60% eosinophils. An endomyocardial biopsy revealed eosinophilic endomyocarditis with granuloma, rare giant cells, and no vasculitis, microorganisms, or malignancy. Her presentation met the criteria for either hypereosinophilic syndrome or eosinophilic granulomatosis with polyangitis. Molecular genetic analysis was negative for myelodysplastic syndrome panel/ Platelet Derived Growth Factor Receptor Beta (PDGFRB) (5q32)/Fibroblast Growth Factor Receptor 1 (FGFR1) Fluorescence In Situ Hybridization (FISH), Feline McDonough Sarcoma-related Tyrosine Kinase 3 (FLT3) Internal Tandem Duplication (ITD) mutation, Calregulin (CALR) exon 9 mutation, and T-cell gene rearrangement/polymerase chain reaction. Bone marrow biopsy revealed a mildly hypocellular marrow with multilineage hematopoiesis,+ megakaryocyte dysplasia, and focal eosinophilia. No excess blasts, no monotypic B-cell population, and no discrete pan T-cell aberrancies were found. Bone marrow cytogenetic studies showed a normal signal pattern for myeloproliferative neoplasms panel/Sec1 Family Domain Containing 2 (SCFD2)-Ligand of Numb Protein-X (LNX)-Platelet-derived Growth Factor Receptor Alpha (PDGFRA) fluorescence in situ hybridization with a normal karyotype of 46 XX. Next-generation sequencing, however, was positive for the JAK2-V617F mutation, a rare molecular abnormality in hypereosinophilic syndrome. The prevalence ranges from approximately 0% to 4%. The JAK2 point mutation leads to aberrant tyrosine phosphorylation and increased cytokine activation. The case demonstrates the complexity and challenging nature of advanced diagnostic opportunities in hypereosinophilia and the potential use, in select subsets, of targeted treatments such as tyrosine kinase inhibitors.