VEXAS syndrome is a rare entity secondary to UBA1 gene mutations, located on the X chromosome. This mutation generates, as a consequence, a characteristic vacuolation on haematopoietic stem-cells. It is characterized by multiple autoinflammatory and haematologic manifestations, which respond and end up being dependent on corticosteroid treatment. In this publication we present a 2-case series diagnosed at our hospital and make a brief literature review of the published evidence so far.
In this study, we aimed to evaluate LIF levels and its possible relationship with disease activity in patients with Takayasu's (TAK) and Giant cell arteritis (GCA) patients.
23 Takayasu's arteritis, 9 Giant cell arteritis patients and 25 healthy volunteers were included in the study. Serum LIF levels were measured ELISA.
The mean age of Giant cell arteritis patients was statistically significantly higher than the other groups (p < 0.001). The rate of women was found to be higher in Takayasu's arteritis (p = 0.021). When healthy control, patients with GCA and Takayasu arteritis were compared, there was a difference in LIF values (p = 0.018). In subgroup analyzes, LIF values were found to be higher in GCA patients compared to healthy controls (p < 0.05). There was no statistically significant correlation between LIF and CRP (Rho = −0.038, p = 0.778), ESR (Rho = 0.114, p = 0.399) and ITAS (Rho = −0.357, p = 0.094). While CRP was statistically significantly higher in patients with disease activity (p = 0.003), there was no statistically significant difference between patients in terms of ESR and LIF values. While there was a statistically significant relationship between CRP (OR = 1.19 [1.03–1.37], p = 0.018) and disease activity in univariate analyses, no statistically significant variable was found in multivariable analyses.
LIF values were significantly higher in patients with Giant cell arteritis compared to healthy controls.
Interstitial lung disease is a leading cause of mortality in patients with systemic sclerosis. Currently, there is a lack of consensus regarding screening, rescreening, diagnosis, and follow-up practices in interstitial lung disease associated with systemic sclerosis (SSc-ILD) in Colombia.
A structured survey focused on clinical practices in patients with SSc-ILD was conducted. Members of the Asociación Colombiana de Neumología y Cirugía de Tórax (Asoneumocito) and the Asociación Colombiana de Reumatología (Asoreuma) were invited to participate from March 2023 to May 2023.
We surveyed 51 pulmonologists and 44 rheumatologists. Overall, 51.6% reported having access to multidisciplinary team discussion in ILD. Among the 95 participants, 78.9% would routinely perform a high-resolution computed tomography scan of the chest once a diagnosis of systemic sclerosis was established. This practice is more frequent among rheumatologists (84.1%) than among pulmonologists (74.5%). Approximately half of the participants would rescreen patients annually with computed tomography scan (56.8%) if baseline images were negative.
Spirometry (81.1%), diffusing capacity of the lung for carbon monoxide (80.0%), and 6-min walk test (55.8%) were the most frequently performed tests upon diagnosis of systemic sclerosis. During follow-up, participants would consider repeating pulmonary function tests mostly every 6 months.
Screening of SSc-ILD is high among pulmonologists and rheumatologists. Decision-making on diagnosis and follow-up is similar between specialties, but there are variations in their frequency and indications. Further research is needed to evaluate how to adapt recommendations for assessing SSc-ILD in different settings.
To design a care protocol in Chronic Inflammatory Arthritis during the pre-conceptional period, pregnancy, postpartum and lactation. This protocol aims to be practical and applicable in consultations where patients with chronic inflammatory rheumatological diseases are treated, thus helping to better control these patients. Likewise, recommendations are offered on when patients could be consulted/referred to a specialized center by the physician.
A multidisciplinary panel of expert physicians from different specialties identified the key points, analyzed the scientific evidence, and met to develop the care protocol.
The recommendations prepared have been divided into three blocks: rheumatology, gynecology and pediatrics. The first block has been divided into pre-pregnancy, pregnancy and postpartum visits.
This protocol tries to homogenize the follow-up of the patients from the moment of the gestational desire until the year of life of the infants. It is important to perform tests in patients of childbearing age and use drugs compatible with pregnancy. If appropriate, the patient should be referred to specialized units. Multidisciplinarity (rheumatology, gynecology and pediatrics) is essential to improve the control and monitoring of these patients and their offspring.
The Colegio Mexicano de Reumatología (CMR) is a corporation whose brand has two elements—image and identity—that differentiate it from other corporations. We evaluated aspects of CMR's corporate image and identity.
To assess corporate image, we designed a survey using proof-of-concept and discrete-choice-experiments approaches. It assessed which definition (orthopedist, rheumatologist, or rehabilitator) was most meaningful in four pain scenarios in healthy adults from the country's Western region.
We used discourse analysis and five readability indices of the CMR website to assess corporate identity.
In total, 700 respondents were included. For every rheumatologist chosen in the hand scenario, respondents chose 1.13 orthopedists and 0.70 rehabilitators. For every rheumatologist chosen in the knee scenario, respondents chose 2.36 orthopedists and 0.64 rehabilitators, whereas 0.85 orthopedists and 0.58 rehabilitators were chosen in the arthritis scenario. Only 38% of the respondents preferred the CMR's definition of a rheumatologist to describe a rheumatologist.
The younger age group preferred orthopedists to rheumatologists (50% vs. 31%, p < 0.001). In the arthritis scenario, the choice of rheumatologist increased from 27% in the elementary school group to 49% in the university group (p < 0.001). Mother was the most influential in healthcare seeking.
The discursive analysis revealed that the CMR is positioned as a “we” restricted to “colleagues;” the patient did not have agentive representation. The semiotic structure of the CMR's mission/vision was deemed imprecise and lacking in statements of value and purpose; the readability scores indicated that the text was challenging and dry.
The CMR's corporate image does not differentiate it from other health providers. CMR's identity seems ambiguous with restricted directionality. It seems pertinent to redefine the CMR.
Hydroxychloroquine (HCQ) is the first-line treatment for systemic lupus erythematosus (SLE); however, there is heterogeneity in its clinical use. This consensus aims to bridge the gap in SLE treatment by providing practical and valuable recommendations for health professionals.
The methodology used is based on a systematic literature review and a nominal group technique (NGT). A ten-member scientific committee formulated eight clinically relevant questions. First, a systematic review was conducted to identify the available evidence, which the scientific committee evaluated to developed recommendations based on their expertise, achieving consensus through NGT.
1673 titles and abstracts were screened, and 43 studies were included for meeting the inclusion criteria. The scientific committee established 11 recommendations for HCQ use in initiation, maintenance, and monitoring, considering benefits and potential adverse effects of HCQ. Unanimous agreement was achieved on all recommendations.
The available evidence supports HCQ's effectiveness and safety for SLE. Individualized assessment of the initial HCQ dose is important, especially in situations requiring dose reduction or discontinuation. This risk–benefit assessment, specifically focusing on the balance between retinal toxicity and the risk of SLE relapse, should guide decisions regarding medication withdrawal, considering disease activity, risk factors, and HCQ potential benefits. Close monitoring is essential for optimal disease management and minimize potential risks, such as QT prolongation or retinal toxicity.
A subset of gout patients developed persistent joint pain after flares. Analysis of this clinical phenomenon may shed further light on the factors related to worsening gout and even provide clues to its pathogenesis.
We analyzed the clinical, laboratory, and ultrasound data of gout patients to explore the associations of these data with persistent joint pain after gout flares.
A total of 1029 gout patients were included: 182 (17.7%) patients with persistent joint pain and 847 (82.3%) patients with nonpersistent joint pain. Patients with persistent joint pain had more total involved joints, more gout flares in the past year, and more joints with simultaneous gout flares (P < 0.01). Among the ultrasound-detected lesions, patients with persistent joint pain had a higher incidence of tophus (36.4% vs. 21.1%) and bone erosion (18.6% vs. 8.6%) (P < 0.05). Higher UA and lower TBil were found in patients with persistent joint pain (P < 0.001). Hypertension (54.9% vs. 38.7%) and metabolic syndrome (58.8% vs. 46.4%) were both more frequent in patients with persistent joint pain (P < 0.05). TBil was negatively correlated with the incidence of persistent joint pain (P < 0.001, r = −0.190), UA values (P < 0.001, r = −0.125), and metabolic syndrome scores (P < 0.001, r = −0.192). A correlation curve was fitted using LOESS (locally weighted region).
Persistent joint pain after gout flares is a marker of increased disease burden in gout. The significance of the level of total bilirubin for the exacerbation of gout deserves further study.
Many patients diagnosed with rheumatoid arthritis (RA) report relief of symptoms after consuming certain foods. Diet plays a vital role in rheumatoid arthritis-related inflammation regulation. This study investigates the relationship between dietary inflammation index (DII) scores and RA disease activity.
Forty-one RA patients were enrolled in the study. The general inflammatory index of the diet was analyzed by recording the 24-h food consumption of the patients, and the nutrients were analyzed using the Nutrition Information Systems Package Program. Dietary inflammatory indices were calculated for each patient using the patients’ macro and micronutrient intake levels. RA disease activity was assessed using the Disease Activity Score-28 (DAS-28).
The DAS-28 score was lower in the anti-inflammatory diet group compared to the pro-inflammatory diet group (p = 0.163). A weak but significant relationship was found between diet inflammation index score and DAS-28 (r = 0.3468, p = 0.0263). The effect of the dietary inflammatory index on the DAS-28 was 12.02%. Dietary iron, vitamin C, niacin, and magnesium intakes were statistically significantly higher in the quartile group that received an anti-inflammatory diet than in the quartile group that received a pro-inflammatory diet. The intake of some micronutrients, such as iron, zinc, magnesium, and folic acid, was significantly lower than the recommended values in all RA quartile groups.
Our results suggest that reducing inflammation through the diet may have a weak but significant effect in controlling disease activity in RA patients.
To describe the impact of the COVID-19 on the psychosocial health of patients with rheumatoid arthritis (RA), spondyloarthritis (SpA), and systemic lupus erythematosus (SLE).
Longitudinal observational study of a series of patients with rheumatic disease.
The main outcome measure was impairment of the ability to participate in social activities, as measured using the PROMIS-APS instrument Short Form-8a. We evaluated social activities in various settings and performed a multivariate analysis to study the association between worsening of social participation during the COVID-19 pandemic and implicated factors.
One hundred and twenty-five patients had completed the prospective follow-up: 40 with AR (32%), 42 with SpA (33.6%), and 43 with SLE (34.4%). Overall, poorer mean PROMIS scores were recorded after the COVID-19 pandemic for: satisfaction with social roles (p = 0.029), depression (p = 0.039), and ability to participate in social activities (p = 0.024). The factors associated with ability to participate in social activities after the COVID-19 pandemic were older age (β = −0.215; p = 0.012), diagnosis of SLE (β = −0.203; p = 0.015), depression (β = −0.295; p = 0.003) and satisfaction with social roles (β = 0.211; p = 0.037).
The ability to participate in social activities after the COVID-19 pandemic is affected in patients with rheumatic disease, especially in SLE.
There is growing interest in the potential of telemedicine (TM) as an alternative to physical consultation. Although numerous studies prove the benefits of TM in rheumatology, there are no recommendations on its implementation in Spain. The aim of this study was to analyze the application of TM in rheumatology consultations in Spain.
Qualitative, cross-sectional, multicenter study with Delphi methodology in two rounds of queries. A structured ad hoc questionnaire was designed that included statements on teleconsultation, nursing teleconsultation, telecare, telerehabilitation, teleradiology, telehealth tele-education, main barriers, advantages and disadvantages of telehealth tele-education and TM in rheumatoid arthritis. The participants were rheumatology specialists practicing in Spain.
The participating rheumatologists (N = 80) had a mean age of 42.4 (±9.0) years, with 12.6 (±8.4) years of experience. Some of the aspects of TM that obtained the greatest consensus were: TM is useful for follow-up of some patients, to help determine if a face-to-face consultation is necessary, or to assist patients with rheumatoid arthritis if they present low activity or in remission; certain patients, such as those in their first consultation or those who present digital barriers or cognitive deterioration, should be seen face-to-face; TM presents some technical and patient access barriers; TM can be useful in nursing and in continued medical education.
TM can be beneficial for the treatment and follow-up of patients with rheumatic diseases, as well as for alleviating the face-to-face care burden in rheumatology.
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