scholarly journals Therapeutic options for patients with rare rheumatic diseases: a systematic review and meta-analysis

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Tim T. A. Bender ◽  
Judith Leyens ◽  
Julia Sellin ◽  
Dmitrij Kravchenko ◽  
Rupert Conrad ◽  
...  
Keyword(s):  
Systematic Review ◽  
Systemic Sclerosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Behcet's Disease ◽  
Randomized Controlled ◽  
Anca Associated Vasculitis

Abstract Background Rare diseases (RDs) in rheumatology as a group have a high prevalence, but randomized controlled trials are hampered by their heterogeneity and low individual prevalence. To survey the current evidence of pharmacotherapies for rare rheumatic diseases, we conducted a systematic review and meta-analysis. Randomized controlled trials (RCTs) of RDs in rheumatology for different pharmaco-interventions were included into this meta-analysis if there were two or more trials investigating the same RD and using the same assessment tools or outcome parameters. The Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, and PUBMED were searched up to April 2nd 2020. The overall objective of this study was to identify RCTs of RDs in rheumatology, evaluate the overall quality of these studies, outline the evidence of pharmacotherapy, and summarize recommended therapeutic regimens. Results We screened 187 publications, and 50 RCTs met our inclusion criteria. In total, we analyzed data of 13 different RDs. We identified several sources of potential bias, such as a lack of description of blinding methods and allocation concealment, as well as small size of the study population. Meta-analysis was possible for 26 studies covering six RDs: Hunter disease, Behçet’s disease, giant cell arteritis, ANCA-associated vasculitis, reactive arthritis, and systemic sclerosis. The pharmacotherapies tested in these studies consisted of immunosuppressants, such as corticosteroids, methotrexate and azathioprine, or biologicals. We found solid evidence for idursulfase as a treatment for Hunter syndrome. In Behçet’s disease, apremilast and IF-α showed promising results with regard to total and partial remission, and Tocilizumab with regard to relapse-free remission in giant cell arteritis. Rituximab, cyclophosphamide, and azathioprine were equally effective in ANCA-associated vasculitis, while mepolizumab improved the efficacy of glucocorticoids. The combination of rifampicin and azithromycin showed promising results in reactive arthritis, while there was no convincing evidence for the efficacy of pharmacotherapy in systemic sclerosis. Conclusion For some diseases such as systemic sclerosis, ANCA-associated vasculitis, or Behcet's disease, higher quality trials were available. These RCTs showed satisfactory efficacies for immunosuppressants or biological drugs, except for systemic sclerosis. More high quality RCTs are urgently warranted for a wide spectrum of RDs in rheumatology.

scholarly journals The Use of Biologic Agents in the Treatment of Oral Lesions due to Pemphigus and Behcet's Disease: A Systematic Review

2020 ◽  
Vol 1 (1) ◽  
pp. 14-23
Author(s):  
Gerald E. Davis II ◽  
George Sarandev ◽  
Alexander T. Vaughan ◽  
Kamal Al-Eryani ◽  
Reyes Enciso
Keyword(s):  
Systematic Review ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Biologic Agents ◽  
Risk Of Bias ◽  
Cochrane Library ◽  
Behcet's Disease ◽  
Randomized Controlled

Background: Current treatments for pemphigus and Behcet's disease, such as corticosteroids, have long-term serious adverse effects. Objective: The objective of this systematic review was to evaluate the efficacy of biologic agents (biopharmaceuticals manufactured via a biological source) on the treatment of intraoral lesions associated with pemphigus and Behcet's disease compared to glucocorticoids or placebo. Methods: PubMed, Web of Science, Cochrane Library, and EMBASE were searched for randomized controlled studies up to January 2019. Bias was assessed with the risk of bias tool. Results: Out of 740 references retrieved, only four randomized controlled trials (RCTs) were included, comprised of a total of 158 subjects (138 pemphigus and 20 Behcet's disease). All studies were assessed at high risk of bias. Heterogeneity of data prevented the authors from performing a meta-analysis. Infliximab or rituximab with short-term prednisone showed higher safety and lowered cumulative prednisone dose than prednisone alone in the treatment of pemphigus. Subcutaneous injection of etanercept provided 45% of patients free of ulcers compared to 5% in the placebo group in one study with Behcet's disease; however, no difference was found in pemphigus patients. Conclusion: Though biological agents alone or in combination with prednisone showed favorable results in three RCTs compared to prednisone alone or placebo, a meta-analysis could not be undertaken due to high heterogeneity. Results are inconclusive, and larger, well-designed RCTs are needed.

scholarly journals Cardiac Function and Diastolic Dysfunction in Behcet’s Disease: A Systematic Review and Meta-Analysis

2016 ◽  
Vol 2016 ◽  
pp. 1-11 ◽  
Author(s):  
Fawad Aslam ◽  
Salman J. Bandeali ◽  
Cynthia Crowson ◽  
Mahboob Alam
Keyword(s):  
Systematic Review ◽  
Diastolic Dysfunction ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Aortic Diameter ◽  
Behcet's Disease ◽  
Reduced Ejection Fraction ◽  
Echocardiographic Parameters

Background. Cardiovascular involvement in Behcet’s disease (BD) is reported and has variable manifestations. It is not clear if diastolic dysfunction (DD) is increased in BD. Our objective was to evaluate the existing literature to determine if cardiac dysfunction, particularly DD, was more prevalent in these patients.Methods. A systematic review and meta-analysis of the available studies analyzing the echocardiographic findings in BD was conducted using a random-effects model. Mean differences were used to calculate the effect sizes of the echocardiographic parameters of interest.Results. A total of 22 studies with 1624 subjects were included in the analysis. Patients with BD had statistically significantly larger mean left atrial dimension (0.08,p=0.0008), greater aortic diameter (0.16,p=0.02), significantly reduced ejection fraction (−1.08,p<0.0001), significantly prolonged mitral deceleration time (14.20,p<0.0001), lowerE/Aratio (−0.24,p=0.05), and increased isovolumetric relaxation time (7.29,p<0.00001).Conclusion.DD is increased in patients with BD by the presence of several echocardiographic parameters favoring DD as compared to controls. The meta-analysis also identified that LA dimension is increased in BD patients. EF has also been found to be lower in BD patients. Aortic diameter was also increased in BD patients as compared to controls.

scholarly journals Herbal Medicine for Behçet’s Disease: A Systematic Review and Meta-Analysis

Nutrients ◽  
2020 ◽  
Vol 13 (1) ◽  
pp. 46
Author(s):  
Ji Hee Jun ◽  
Tae Young Choi ◽  
Hye Won Lee ◽  
Lin Ang ◽  
Myeong Soo Lee
Keyword(s):  
Systematic Review ◽  
Herbal Medicine ◽  
Behçet’S Disease ◽  
Methodological Quality ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Risk Of Bias ◽  
Behcet's Disease ◽  
Assessment Development

Patients with Behçet’s disease often use complementary and alternative medicine for treating their symptoms, and herbal medicine is one of the options. This systematic review provides updated clinical evidence of the effectiveness of herbal medicine for the treatment of Behçet’s disease (BD). We searched eleven electronic databases from inception to March 2020. All randomized controlled trials (RCTs) or quasi-RCTs of BD treatment with herbal medicine decoctions were included. We used the Cochrane Handbook for Systematic Reviews of Interventions to assess the risk of bias and the grading of recommendations assessment, development and evaluation (GRADE) approach to assess the certainty of evidence (CoE). Albatross plot was also used to present the direction of effect observed. Eight studies were included. The risk of bias was unclear or low. The methodological quality was low or very low. Seven RCTs showed significant effects of herbal medicine on the total response rate (Risk ratio, RR 1.26, 95% CI 1.09 to 1.45, seven studies, very low CoE). Four RCTs showed favorable effects of herbal medicine on the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level compared with drug therapy. Herbal medicine favorably affected the ESR (MD −5.56, 95% CI −9.99 to −1.12, p = 0.01, I2 = 96%, five studies, very low CoE). However, herbal medicine did not have a superior effect on CRP. Two RCTs reported that herbal medicine significantly decreased the recurrence rate after three months of follow-up (RR 0.23, 95% CI 0.09 to 0.63, two studies, low CoE). Our findings suggest that herbal medicine is effective in treating BD. However, the included studies had a poor methodological quality and some limitations. Well-designed clinical trials with large sample sizes are needed.

scholarly journals AB0385 THE EFFECTIVENESS OF THE ANTI-TUMOR NECROSIS FACTOR THERAPY INFLIXIMAB IN NEURO-BEHCET’S DISEASE: A SYSTEMATIC REVIEW AND META-ANALYSIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1220.2-1220
Author(s):  
R. Hamdy Abdellatif Mohammed ◽  
Y. Woldeamanuel
Keyword(s):  
Systematic Review ◽  
Behçet’S Disease ◽  
Effect Size ◽  
Behcet’S Disease ◽  
Disease Duration ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Research Question ◽  
Behcet's Disease ◽  
Study Heterogeneity

Background:The use and benefit of biologic targeted therapy in the treatment of refractory manifestations of behcet’s syndrome remain unclearly defined due to the relative paucity of published research. Neuro-behcet’s disease represents a challenging clinical presentation. The lack of identified effective strategies in the management of refractory neuro-behcet’s adds to the disease burden and contributes to lack of tight control. [1-3]Objectives:The aim of this systematic review and meta-analysis is to examine the effect of anti-TNF therapy on refractory NBD by performing a systematic review and meta-analysis of the previously published studies assessing the effectiveness of anti-TNF therapy in patients with BD having refractory or recurrent neurological manifestations.Methods:Authors used the PICO Model (P= population, I= intervention, C= comparator, O= outcome) used for designing the research question. The PRISMA statement was used for developing the study protocol search methodology. Literature searches were done for articles in published in English language only from January 2000 till January 2020 (considering the first till the last available publication that addresses the research question and satisfied eligibility criteria). The study was registered on the Prospero PROSPERO website for systematic reviews and meta-analysis. Random-effects meta-analysis was performed. Inter-study heterogeneity was explored using I2 statistics. Cumulative meta-analysis was conducted to assess temporal trend for accumulating evidence from accruing published studies. Meta-regression was used to analyze possible confounders.Authors of the current systematic review and meta-analysis worked on a simple score “Hamdy and Woldeamanuel simple response score, 2020”, the aim behind the score was to incorporate standard evaluation parameters for assessment of responses driven by the measures used in each study. The authors used numerical values to grade the pattern of response in each parameter considered attempting to standardize the quantification of the responses and minimize tendency towards overestimation. The score included clinical and radiographic points rated on a scale of 5.Results:Twenty-one studies involving 64 patients were included, 52 male patient and 12 females at a ratio of 4.3:1, with a mean age of 38 .21 years, mean disease duration of 84.76 5 months. Effect size analysis showed that 59 out of 64 patients i.e. 93.7% of the treated patients with neuro-behcet’s disease in the analysis were responders to infliximab therapy (CI 88% - 99.3%). There was no significant inter-study heterogeneity (I2 = 0%, p = 0.744). Cumulative analysis showed accumulating evidence favoring increasing effectiveness over the last 20 years. There was no statistically significant confounding of infliximab effect size by age (p = 0.89), sex ratio (p = 0.29), and disease duration (p = 0.67).Conclusion:In this systematic review and meta-analysis Infliximab showed a strong therapeutic effectiveness in the treatment of refractory neuro-behcet’s disease. There is an increasing trend of effectiveness observed in the last two decades which may be due to improved neuro-behcet’s diagnostic accuracy.References:[1]Uygunoğlu U, Siva A. Behçet’s Syndrome and Nervous System Involvement. Curr Neurol Neurosci Rep. 2018;18(7):35. Published 2018 May 23. doi:10.1007/s11910-018-0843-5.[2]Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M. Close Association of HLA-Bw51 With Behçet’s Disease. Arch Ophthalmol. 1982; 100(9):1455–1458. doi:10.1001/archopht.1982.01030040433013.[3]Kalra, S., Silman, A., Akman-Demir, G., et al. Diagnosis and management of Neuro-Behçet’s disease: international consensus recommendations. Journal of neurology, 2014; 261(9): 1662–1676. https://doi.org/10.1007/s00415-013-7209-3.Figure 1a. Forest plot summarizing the meta-analysis results on the effectiveness of infliximab to treat Neurobehcet disease. Overall, 94% of patients responded to infliximab.Disclosure of Interests:None declared

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