rheumatological disease
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Author(s):  
Marta Elizabeth Osorio Fleitas ◽  
Blas Antonio Medina Ruíz ◽  
Blás Agustín Medina Izcurdia ◽  
Joaquín Julián Medina Izcurdia

Forestier Syndrome is a rheumatological disease of unknown origin, which consist in calcification of different areas of the body, predominantly at the level of the anterior common vertebral ligament, where osteophytes capable of producing varied symptoms of variable intensity are formed. Normally asymptomatic, although, when it affects the cervical spine, the most common symptom is dysphagia. A case of Forestier syndrome is presented who consulted for dysphagia in the Head and Neck Surgery Unit of the Otolaryngology Service of the Institute of Social Prevision, Asuncion, Paraguay. The semiology allowed to observe a submucosal bulge in the posterior wall of the oropharynx of 1,5 cm in diameter, which could be evaluated and confirmed by tomography. Due to the low intensity of the symptoms and little impact on the general state, it was decided to carry out a conservative treatment with good results, after two years of clinical follow-up. In these cases, surgery occupies a secondary place, either before the failure of this conservative behavior or the progression of the symptoms.


Acta Medica ◽  
2021 ◽  
pp. 1-8
Author(s):  
Berkan Armağan ◽  
Bayram Farisoğulları ◽  
Hakan Oral ◽  
Levent Kılıç ◽  
Şule Apraş Bilgen ◽  
...  

Objective: Intravenous immunoglobulin is an alternative therapeutic agent that can be used off-label in many autoimmune rheumatological diseases. The aim of this study is to evaluate the autoimmune rheumatological diseases characteristics in which intravenous immunoglobulin therapy is used and the efficacy and safety of this therapy. Methods and Methods: We performed a retrospective review of 133 patients with autoimmune rheumatological disease who received at least 1 course of intravenous immunoglobulin treatment at Hacettepe University Rheumatology Outpatient Clinic between January 2013 and December 2020. The autoimmune rheumatological disease demographic and clinical features, organ involvements, treatment phases (primary-secondary or infection), treatment responses and adverse effects were evaluated. Results: A total of 79% (n=105) patients were female and the mean±SD age was 45.5±16.9 years. The most common underlying rheumatic diseases were systemic lupus erythematosus (35%, n=47) and dermatomyositis/polymyositis (35%, n=47). Intravenous immunoglobulin therapy was most commonly used for resistant/relapsed myositis and haematological involvement. The median (IQR) intravenous immunoglobulin treatment course was 6.5 (13) and the duration of intravenous immunoglobulin treatment was 10.8 (24) months. Although it is used as second-line therapy in 77% of patients, complete clinical response was observed in 32% and partial response in 47%. There was a significant reduction in the median (IQR) steroid doses (methylprednisolone or equivalent dose) patients received from baseline after intravenous immunoglobulin treatment [30 (33) vs 8 (12), p<0.0001]. It was observed that the use of conventional disease-modifying antirheumatic drugs decreased after intravenous immunoglobulin treatment and the use of rituximab increased. Adverse effects associated with intravenous immunoglobulin treatment (10%) and discontinuation (4%) were found to be very low. Conclusion: Intravenous immunoglobulin treatment was commonly given in systemic lupus erythematosus and dermatomyositis/polymyositis patients because of hematological involvement and resistant/relapsed myositis in our study, respectively. Although it is mainly the second-line treatment, two-thirds of the patients achieved a complete/partial response. Side effects and related discontinuation due to intravenous immunoglobulin treatment are very few.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Marzieh Keshtkarjahromi ◽  
Sumit Chhetri ◽  
Amulya Balagani ◽  
Umm-ul-Banin B. Tayyab ◽  
Christopher J. Haas

Abstract Background Macrophage activation syndrome (MAS) is a rare multiorgan system disorder that may present as a fatal complication of underlying rheumatological disease, including dermatomyositis. Case presentation Here, we report the case of a 65-year-old Caucasian female with a history of psoriasis and a recent diagnosis of Coronavirus disease 2019 (COVID-19) who presented with progressive generalized weakness, joint pains, an erythematous rash, shortness of breath, and weight loss. She was ultimately diagnosed with biopsy-confirmed melanoma differentiation-associated protein 5 (MDA5)-positive dermatomyositis complicated by MAS, requiring intravenous immunoglobulin and high-dose methylprednisolone. Conclusions This report serves as a clinical reminder of the rare, yet clinically relevant association between MDA5-positive dermatomyositis and MAS, as well as highlights the potential contribution of other immune system activating diseases, such as COVID-19, associated with a cytokine storm and hyperinflammatory state.


2021 ◽  
Vol 50 (12) ◽  
pp. 873-878
Author(s):  
Daniel Mazzoni ◽  
Paul Kubler ◽  
Jim Muir

Author(s):  
Adrian M Shields ◽  
Srinivasan Venkatachalam ◽  
Salim Shafeek ◽  
Shankara Paneesha ◽  
Mark Ford ◽  
...  

Abstract B cell depleting agents are amongst the most commonly used drugs to treat haemato-oncological and autoimmune diseases. They rapidly induce a state of peripheral B cell aplasia with the potential to interfere with nascent vaccine responses, particularly to novel antigens. We have examined the relationship between B cell reconstitution and SARS-CoV-2 vaccine responses in two cohorts of patients previously exposed to B cell depleting agents: a cohort of patients treated for haematological B cell malignancy and another treated for rheumatological disease. B cell depletion severely impairs vaccine responsiveness in the first 6 months after administration: SARS-CoV-2 antibody seroprevalence was 42.2% and 33.3% in the haemato-oncological patients and rheumatology patients respectively and 22.7% in patients vaccinated while actively receiving anti-lymphoma chemotherapy. After the first 6 months, vaccine responsiveness significantly improved during early B cell reconstitution, however, the kinetics of reconstitution was significantly faster in haemato-oncology patients. The AstraZeneca ChAdOx1 nCoV-19 vaccine and the Pfizer BioNTech 162b vaccine induced equivalent vaccine responses, however shorter intervals between vaccine doses (&lt;1m) improved the magnitude of the antibody response in haeamto-oncology patients. In a subgroup of haemato-oncology patients, with historic exposure to B cell depleting agents (&gt;36m previously) vaccine non-responsiveness was independent of peripheral B cell reconstitution. The findings have important implications for primary vaccination and booster vaccination strategies in individuals clinically vulnerable to SARS-CoV-2.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Linda Silke ◽  
Othman Kirresh ◽  
Jackie Sturt ◽  
Heidi Lempp

Abstract Background Patients with Rheumatoid Arthritis (RA) may experience psychological distress (depression, anxiety) in addition to their physical symptoms. People with RA may also experience disease-specific distress (DSD), related to the specific burden of living with their life-long condition. DSD is a patient reported outcome in several long-term conditions, including type 1 and 2 diabetes. The aims of this study were to determine whether DSD is experienced by people with RA, and if so, develop a Patient Reported Outcome Measure (PROM) to assess for DSD in people with RA. Methods A five-phased qualitative study was conducted which consisted of a secondary data analysis of 61 interviews of people with rheumatological disease (Phase 1), validation of findings via a Patient and Public Involvement (PPI) group of people with RA (n = 4) (Phase 2), item generation for a PROM (Phase 3) and establishing face and content validity of the PROM via PPI group (n = 4) and individual cognitive interviews (n = 9) of people with RA respectively (Phase 4 and 5). The final PROM was presented at a Patient Education Evening for patients with long-term rheumatological conditions, including RA, and carers. Results Five themes of rheumatological disease distress emerged from Phase 1, which were validated in the Phase 2 PPI group. After Phases 3–5, the Rheumatoid Arthritis Distress Scale (RADS) was formed of 39 items and 3 supplementary questions. Overall participants reported the content of the RADS to be clear and relevant, and that DSD is a valid concept in RA, distinct from other entities like clinical depression or anxiety. Conclusions DSD appears to be an important concept in RA. The 39-item RADS demonstrates acceptable face and content validity in this patient group. Further psychometric testing is needed. The RADS may be a useful tool for healthcare professionals to identify RA distress.


2021 ◽  
Vol 12 ◽  
Author(s):  
Ju Li ◽  
Zhongyuan Zhang ◽  
Xinhua Wu ◽  
Jie Zhou ◽  
Deqian Meng ◽  
...  

Background: Adalimumab, golimumab, infliximab, certolizumab, and etanercept are five anti-tumor necrosis factor (anti-TNF) medicines that have been approved for use in rheumatology. Apart from their well-established therapeutic usefulness, -it is unclear to what extent -they are linked to an increased risk of various side effects. The present meta-analysis was carried out to assess the risk of infection and other side effects after anti-TNF- α for the treatment of rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis.Methods: We searched PubMed, Cinahl (via Ebsco), Scopus, and Web of Sciences databases for trials comparing anti-TNF medications to placebo or no therapy in adult patients with rheumatoid arthritis, psoriatic arthritis, or ankylosing spondylitis from August 2006 to August 2020. A total of 23 articles were used for meta-analysis. The Cochrane Collaboration’s risk of bias tool was used to assess the methodological quality of the included studies. In addition, a random-effects model was used to calculate the pooled odds ratio, and Forest plots were constructed to determine the risk of infections and cancer following the use of anti-TNF treatment.Results: Treatment with anti-TNFα agents resulted in an increase in the risk of serious infections (OR: 1.72, 95% CI: 1.56–1.90, p &lt; 0.00001) and an increase in cancer risk (OR: 1.36, 95% CI: 1.20–1.53, p &lt; 0.00001) whereas the risk of developing tuberculosis was not significantly different with anti-TNFα agents versus those without treatment with anti-TNFα agents (OR: 2.55, 95% CI: 0.40–16.23, p = 0.32) although the number of studies is limited to make a definitive conclusion. The risk of bias of the included studies was unclear to high across most domains, and there was evidence of publication bias for most outcomes.Conclusion: The present meta-analysis suggests an increased risk of infectious adverse events, including overall adverse events and cancer following anti-TNFα treatment, whereas the risk of tuberculosis was not significantly different. Although anti-TNF agents have shown promise to treat inflammatory conditions, their use should be balanced by the risk-benefit ratio as suggested by the meta-analysis.


2021 ◽  
Vol 78 (4) ◽  
pp. 485-495
Author(s):  
yanfang lin ◽  
Songsen SU ◽  
HONG ZHOU ◽  
LILI CAI ◽  
LEI HONG

2021 ◽  
pp. postgradmedj-2021-140754
Author(s):  
Wei Syun Hu ◽  
Cheng Li Lin

PurposeThis is a nationwide-based retrospective study aiming to compare the three different scoring systems (CHA2DS2-VASc, C2HEST and HAVOC scores) in the prediction of atrial fibrillation (AF) in patients with rheumatological disease.MethodsWe used the Fine and Gray model to estimate the risk of AF (subhazard ratio and 95% CI). The predictive accuracy and discriminatory ability of the predictive model were evaluated by receiver operating characteristic (ROC) curve.ResultsAmong the three predictive models, the model using CHA2DS2-VASc score had the better discriminative ability with an ROC of 0.79. The model with C2HEST score had an ROC of 0.78. The discriminative ability of the HAVOC score was 0.77, estimated by ROC.ConclusionWe concluded the CHA2DS2-VASc score has better performance in predicting AF compared with C2HEST score or HAVOC score.


2021 ◽  
pp. 77-79
Author(s):  
Hanan AlObaid ◽  
Laila AlRashed ◽  
Ayat AlHraiz ◽  
Erum Khalid ◽  
Mai AlDhamadi

Introduction: Fibromyalgia is a common rheumatological disease that is difcult to diagnose , because of it's subjective symptoms and decient physician's knowledge of the disease . The purpose of this study is to assess knowledge, attitude and practice of family physicians regarding bromyalgia Methods: The study was a cross sectional with an online survey that was administered to general practitioners and family physicians in Eastern province, responses were obtained from 209 participants, knowledge about bromyalgia, attitude and practice were assessed Results: Regarding knowledge about bromyalgia, 89% of the respondents had poor knowledge. Attitudes revealed that More than half of the respondents (56%) thought that bromyalgia is easy to diagnosed. However, only 12.4% of the medical practitioners agreed that diagnosis of bromyalgia can be conducted in PHC setting and only 16.7% felt condent to use (ACR) criteria. Regarding management, about 27% believed they can manage bromyalgia . Only a quarter (24.9%) said they never manage any cases of bromyalgia. Conclusions: knowledge, attitude and practice regarding bromyalgia are poor which results in delay in the diagnosis and management.To overcome this delays in diagnosis and treatment and improve knowledge about bromyalgia, further education is needed


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