How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review

Behçet syndrome (BS) is a multisystem vasculitis that is most active during young adulthood, causing serious disability and significant impairment in quality of life. Differences in the disease course, severity, and organ involvement between patients, depending on the age at presentation and sex, makes it impossible to determine a single management strategy. The diversity and variability in the outcome measures used in clinical trials in BS makes it difficult to compare the results or inform physicians about the best management strategy for individual patients. There is a large unmet need to determine or develop validated outcome measures for use in clinical trials in BS that are acceptable to researchers and regulatory agencies. We conducted a systematic review to describe the outcomes and outcome measures that have been used in clinical trials in BS. This review revealed the diversity and variability in the outcomes and outcome measures and the lack of standard definitions for most outcomes and rarity of validated outcome tools for disease assessment in BS. This systematic literature review will identify domains and candidate instruments for use in a Delphi exercise, the next step in the development of a core set of outcome measures that are properly validated and widely accepted by the collaboration of researchers from many different regions of the world and from different specialties, including rheumatology, ophthalmology, dermatology, gastroenterology, and neurology.

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Azathioprine therapy in incomplete Behcet syndrome

Archives of Dermatology ◽

10.1001/archderm.110.3.432 ◽

1974 ◽

Vol 110 (3) ◽

pp. 432-434 ◽

Cited By ~ 4

Author(s):

J. Nethercott

Keyword(s):

Azathioprine Therapy ◽

Behçet Syndrome ◽

Behcet Syndrome

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Behcet Syndrome

10.32388/ri64bz ◽

2020 ◽

Author(s):

Keyword(s):

Behçet Syndrome ◽

Behcet Syndrome

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Clinical features and outcomes of pregnant women with COVID-19: a systematic review and meta-analysis

BMC Infectious Diseases ◽

10.1186/s12879-020-05274-2 ◽

2020 ◽

Vol 20 (1) ◽

Cited By ~ 9

Author(s):

Yi-jie Gao ◽

Lei Ye ◽

Jia-shuo Zhang ◽

Yang-xue Yin ◽

Min Liu ◽

...

Keyword(s):

Systematic Review ◽

Pregnant Women ◽

Clinical Features ◽

Meta Analysis

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Clinical features and outcomes of COVID-19 in older adults: a systematic review and meta-analysis

BMC Geriatrics ◽

10.1186/s12877-021-02261-3 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Sunny Singhal ◽

Pramod Kumar ◽

Sumitabh Singh ◽

Srishti Saha ◽

Aparajit Ballav Dey

Keyword(s):

Systematic Review ◽

Older Adults ◽

Critically Ill ◽

Clinical Features ◽

Older Patients ◽

Clinical Characteristics ◽

Meta Analysis ◽

Severe Infection ◽

Factors Affecting ◽

Pooled Prevalence

Abstract Background Few studies have focused on exploring the clinical characteristics and outcomes of COVID-19 in older patients. We conducted this systematic review and meta-analysis to have a better understanding of the clinical characteristics of older COVID-19 patients. Methods A systematic search of PubMed and Scopus was performed from December 2019 to May 3rd, 2020. Observational studies including older adults (age ≥ 60 years) with COVID-19 infection and reporting clinical characteristics or outcome were included. Primary outcome was assessing weighted pooled prevalence (WPP) of severity and outcomes. Secondary outcomes were clinical features including comorbidities and need of respiratory support. Result Forty-six studies with 13,624 older patients were included. Severe infection was seen in 51% (95% CI– 36-65%, I2–95%) patients while 22% (95% CI– 16-28%, I2–88%) were critically ill. Overall, 11% (95% CI– 5-21%, I2–98%) patients died. The common comorbidities were hypertension (48, 95% CI– 36-60% I2–92%), diabetes mellitus (22, 95% CI– 13-32%, I2–86%) and cardiovascular disease (19, 95% CI – 11-28%, I2–85%). Common symptoms were fever (83, 95% CI– 66-97%, I2–91%), cough (60, 95% CI– 50-70%, I2–71%) and dyspnoea (42, 95% CI– 19-67%, I2–94%). Overall, 84% (95% CI– 60-100%, I2–81%) required oxygen support and 21% (95% CI– 0-49%, I2–91%) required mechanical ventilation. Majority of studies had medium to high risk of bias and overall quality of evidence was low for all outcomes. Conclusion Approximately half of older patients with COVID-19 have severe infection, one in five are critically ill and one in ten die. More high-quality evidence is needed to study outcomes in this vulnerable patient population and factors affecting these outcomes.

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Risk Factors, and Clinical and Etiological Characteristics of Ischemic Strokes in COVID-19-Infected Patients: A Systematic Review of Literature

Cerebrovascular Diseases ◽

10.1159/000514267 ◽

2021 ◽

pp. 1-4

Author(s):

Simone Vidale

Keyword(s):

Risk Factors ◽

Systematic Review ◽

Clinical Features ◽

Case Reports ◽

Clinical Severity ◽

Large Artery ◽

Causal Association ◽

Review Of Literature ◽

Increased Risk ◽

Long Time

Background and Purpose: Coronavirus disease 2019 (COVID-19) infection is an ongoing pandemic and worldwide health emergency that has caused important changes in healthcare systems. Previous studies reported an increased risk of thromboembolic events, including stroke. This systematic review aims to describe the clinical features and etiological characteristics of ischemic stroke patients with COVID-19 infection. Method: A literature search was performed in principal databases for studies and case reports containing data concerning risk factors, clinical features, and etiological characteristics of patients infected with COVID-19 and suffering from stroke. Descriptive and analytical statistics were applied. Results: Overall, 14 articles were included for a total of 93 patients. Median age was 65 (IQR: 55–75) years with prevalence in males. Stroke occurred after a median of 6 days from COVID-19 infection diagnosis. Median National of Institute of Health Stroke Scale (NIHSS) score was 19. Cryptogenic (Cry) strokes were more frequent (51.8%), followed by cardioembolic etiology, and they occurred a long time after COVID-19 diagnosis compared with large-artery atherosclerosis strokes (ptrend: 0.03). The clinical severity of stroke was significantly associated with the severity grade of COVID-19 infection (ptrend: 0.03). Conclusions: Ischemic strokes in COVID-19-infected patients were clinically severe, affecting younger patients mainly with Cry and cardioembolic etiologies. Further multicenter prospective registries are needed to better describe the causal association and the effect of COVID-19 infection on stroke.

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Primary and radiation induced skull base osteosarcoma: a systematic review of clinical features and treatment outcomes

Journal of Neuro-Oncology ◽

10.1007/s11060-021-03757-z ◽

2021 ◽

Author(s):

Othman Bin Alamer ◽

Ali S. Haider ◽

Maryam Haider ◽

Navraj S. Sagoo ◽

Faith C. Robertson ◽

...

Keyword(s):

Systematic Review ◽

Skull Base ◽

Treatment Outcomes ◽

Clinical Features ◽

Radiation Induced

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Perioperative management with biologics on severe aortic valve regurgitation caused by Behçet syndrome: the experience from a single center

Therapeutic Advances in Chronic Disease ◽

10.1177/20406223211026753 ◽

2021 ◽

Vol 12 ◽

pp. 204062232110267

Author(s):

Luxi Sun ◽

Jinjing Liu ◽

Xiufeng Jin ◽

Zhimian Wang ◽

Lu Li ◽

...

Keyword(s):

Aortic Valve ◽

Perioperative Management ◽

Perioperative Period ◽

Valve Regurgitation ◽

Aortic Valve Regurgitation ◽

Behçet Syndrome ◽

Reactive Protein ◽

Behcet Syndrome ◽

Sparing Effect ◽

Median Interval

Background: To investigate the efficacy and safety of biologics in the perioperative management of severe aortic valve regurgitation (AR) caused by Behçet syndrome (BS). Methods: We retrospectively analyzed 20 patients with severe AR caused by BS who were all treated with biologics during the perioperative period of cardiac surgeries in our center between February 2016 and October 2020. Results: A total of 20 patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1 ± 8.8 years and a median course of 8 [interquartile range (IQR) 5.25–10.00] years. Before biologic administration, 92.9% of the patients who underwent aortic valve replacement had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics were administered with background glucocorticoids (GCs) and immunosuppressants during the perioperative period for 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR 2.75–4.25) months in 13 cases and within 3 months postoperatively in 9 cases. After a median follow up of 21 (IQR 15–32) months, 2 out of 13 cases (15.4%) preoperatively, and 1 out of 9 cases (11.1%) postoperatively treated with biologics developed PVL, and the rest were event free. The Behçet’s Disease Current Activity Form score improved significantly (7 versus 0, median, p < 0.0001). Decrease of erythrocyte sedimentation rate [25.0 (IQR 11.00–36.25) mm/h versus 6.5 (IQR 4.0–8.8) mm/h, p < 0.001], and C-reactive protein [20.77 (IQR 7.19–29.58) mg/l versus 1.53 (IQR 0.94–2.92) mg/l, p = 0.001] were achieved rapidly and effectively. The GC dosage tapered from 40 (IQR 30–60) mg/d to 10 (IQR 5–11.25) mg/d, p < 0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed. Conclusion: Our study suggests that biologics were effective and well tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had favorable GC- and immunosuppressant-sparing effect.

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