Lymphoid interstitial pneumonia in a patient with systemic lupus erythematosus: Case report and literature review.

Lymphoid interstitial pneumonia (LIP) is a rare form of interstitial pulmonary disease, which has been described in association with a wide range of autoimmune disorders. Although the association of this entity with Sjogren's syndrome is well known, only a few cases are reported in relation to systemic lupus erythematosus (SLE). The aim of this paper is to review the cases reported in literature to date, as well as to describe the characteristics of these patients including the new case presented herein. We will be focusing on the case of a 36-year-old female patient diagnosed with SLE on hydroxychloroquine treatment who develops pleuritic chest pain and progressive dyspnea after 3 years of follow-up. The chest CT scan showed pleural thickening and both multiple and bilateral micronodules. A lung biopsy was also performed, revealing an infiltration of lymphocytes, plasma cells, and histiocytes in the alveolar septa suggestive of LIP. After conducting a review of the literature, we identified seven other cases describing SLE in association with LIP. The majority of them were young women, and LIP tends to appear early in the course of the disease, even as a form of initial presentation in some cases. Symptoms included cough, dyspnea, and pleuritic pain, with the exception of one case which was asymptomatic. It is noteworthy that half of the patients were positive for anti-SSA/anti-SSB autoantibodies, and some of them also met criteria for Sjogren's syndrome. Treatment with steroids and other immunosuppressive agents improved symptoms in all of them.

Single-cell transcriptomic profiling reveals a pathogenic role of cytotoxic CD4+ T cells in giant cell arteritis.

Giant cell arteritis (GCA) is a systemic vasculitis mediated by an aberrant immunological response against the blood vessel wall. Although the pathogenic mechanisms that drive GCA have not yet been elucidated, there is strong evidence that CD4+ T cells are key drivers of the inflammatory process occurring in this vasculitis. The aim of this study was to further delineate the role of CD4+ T cells in GCA by applying single-cell RNA sequencing and T cell receptor (TCR) repertoire profiling to 114.799 circulating CD4+ T cells from eight GCA patients in two different clinical states, active and in remission, and eight healthy controls. Our results revealed an expansion of cytotoxic CD4+ T lymphocytes (CTLs) in active GCA patients, which expressed higher levels of cytotoxic and chemotactic genes when compared to patients in remission and controls. Accordingly, differentially expressed genes in CTLs of active patients were enriched in pathways related to granzyme-mediated apoptosis, inflammation, and the recruitment of different immune cells, suggesting a role of this cell type in the inflammatory and vascular remodelling processes occurring in GCA. CTLs also exhibited a higher clonal expansion in active patients with respect to those in remission. Drug repurposing analysis prioritized maraviroc, which targeted CTLs, as potentially repositionable for this vasculitis. In addition, effector regulatory T cells (Tregs) were decreased in GCA and showed lower expression of genes involved in their suppressive activity. These findings provide further insights into the pathogenic role of CD4+ T cells in GCA and suggest targeting CTLs as a potential therapeutic option.

Adherence to antimalarials and glucocorticoids treatment and its association with self-reported disease activity in systemic lupus erythematosus patients.

OBJECTIVES: We aimed to investigate the rate of non-adherence to antimalarials and glucocorticoids (GCs) and to analyze their potential relationships with sociodemographic characteristics, disease activity and accumulate damage in a cohort of Systemic lupus erythematosus (SLE) patients. METHODS: A cross-sectional study was conducted among 670 patients. The Systemic Lupus Erythematosus Activity Questionnaire (SLAQ) and the Lupus Damage Index Questionnaire (LDIQ) were used to assess disease activity and accumulated damage. RESULTS: The prevalence of non-adherence to antimalarials and GCs were 10.67% and 39.61%. 86.9% of participants indicated that the reason for stopping therapy was the presence of side effects. SLE patients with non-adherence to antimalarials and GCs had significantly higher scores in disease severity (SLAQ) compared to adherence patients (5.03 (2.12) vs 4.39 (2.61); p = .004 and (4.75 (2.29) vs 4.05 (2.78); p ≤ .001). CONCLUSION: Adherence to the treatment indicated in SLE differs from drug to drug. Findings highlight the importance of developing interventions to support adherence and improve outcomes among patients.

Psychological and quality of life effects of vaccination against COVID-19 in patients with systemic autoimmune diseases.

To verify the psychological and quality of life benefits of vaccination against COVID-19 in patients with systemic autoimmune diseases. In this study, levels of psychological stress, psychopathological symptoms, quality of life, and satisfaction with life were compared in patients with systemic autoimmune diseases vaccinated against COVID-19 (n = 132) versus unvaccinated patients (n = 254). To this end, we used the Perceived Stress Scale (PSS), Symptom Checklist-90-Revised (SCL-90-R), EUROQoL-5Q health questionnaire, and Satisfaction with Life Scale (SWLS), respectively. Statistically significant differences were found with better scores in the vaccinated group in the following quality of life dimensions: mobility (p ≤ 0.010), domestic activities (p ≤ 0.004), pain/discomfort (p ≤ 0.001), and anxiety/depression (p≤ 0.005). The scores were also significantly higher in the vaccinated group for the total values of quality of life (p ≤ 0.001), health status self-assessment on the EUROQoL-5Q (p ≤ 0.043), and satisfaction with life (p ≤ 0.015). In addition, the unvaccinated group presented higher scores with clinically pathological levels in depression and psychoticism for somatizations (p ≤ 0.006), depression (p ≤ 0.015), anxiety (p ≤ 0.003), and phobic anxiety (p ≤ 0.001). Finally, participants vaccinated with the complete regimen showed better levels of psychological well-being than those who were not vaccinated or those that had not completed the vaccination regimen. Our results reflect and confirm the positive effects reported elsewhere of the COVID-19 vaccine in autoimmune patients with systemic diseases, both in terms of quality and satisfaction with life as well as psychopathological symptoms and perceived stress. These benefits increased as the patients completed their vaccination schedule.

Clinical course of Covid-19 in a cohort of patients with Behçet disease.

Objective: The implications of Covid-19 in patients with Behçet's disease (BD) are unknown. Patients with BD usually take long-term therapy with therapeutic agents that have been tested in Covid-19 patients. We aimed to assess the prevalence of Covid-19 in a cohort of patients with BD and investigate whether those patients with a long-term treatment with colchicine, tumor necrosis factor inhibitors (TNFi) or glucocorticoids are at reduced or increased prevalence of Covid-19 related clinical outcomes. Methods: A retrospective study was conducted among 244 patients with BD (86.1% females; mean age 43.95 ± 11.11 years). Each participant completed an online questionnaire regarding demographics, medical conditions, dispensed colchicine, TNFi or oral glucocorticoids, Covid-19 infection, clinical symptoms and recovery. Results: The prevalence of Covid-19 infection was 14.75%. Regarding dose of colchicine, the presence of ageusia was lower in patients taking 0.5 mg/day of colchicine compared to those taking 1.5 mg/day (p = 0.021). The prevalence of dyspnea was significantly higher in patients taking TNFi compared with those without therapy (p = 0.032). With regards to oral glucocorticoids, no significant differences were found. Conclusions: The prevalence of Covid-19 among patients with BD seems to be higher than that among the general population in Spain. Continuous TNFi therapy might increase the prevalence of worse clinical outcomes such as dyspnea; oral glucocorticoids and colchicine apparently provided no protection against the Covid-19 related clinical outcomes of patients with BD.

Objetivo: Se desconocen las implicaciones de la Covid-19 en pacientes con enfermedad de Behçet (EB). Los pacientes con EB generalmente tienen tratamiento de larga duración con agentes terapéuticos que se han probado en pacientes con Covid-19. Nuestro objetivo fue evaluar la prevalencia de la Covid-19 en una cohorte de pacientes con EB e investigar si los pacientes con un tratamiento de larga duración con colchicina, inhibidores del factor de necrosis tumoral (TNFi) o glucocorticoides tienen una prevalencia reducida o aumentada en los resultados clínicos de la Covid-19. Métodos: Se realizó un estudio retrospectivo en 244 pacientes con EB (86,1% mujeres; edad media, 43,95 ± 11,11 años). Cada participante completó un cuestionario en línea sobre datos demográficos, afecciones médicas, tratamiento con colchicina, TNFi o glucocorticoides orales, infección por Covid-19, síntomas clínicos y recuperación. Resultados: La prevalencia de la infección por Covid-19 fue del 14,75%. En cuanto a la dosis de colchicina, la presencia de ageusia fue menor en los pacientes que tomaban 0,5 mg/día de colchicina en comparación con los que tomaban 1,5 mg/día (p = 0,021). La prevalencia de disnea fue significativamente mayor en los pacientes que tomaban TNFi en comparación con aquellos sin terapia (p = 0,032). Con respecto a los glucocorticoides orales, no se encontraron diferencias significativas. Conclusiones: La prevalencia de Covid-19 en pacientes con EB parece ser superior a la de la población general en España. La terapia continua con TNFi podría aumentar la prevalencia de peores resultados clínicos como la disnea; los glucocorticoides orales y la colchicina aparentemente no proporcionan protección contra los resultados clínicos relacionados con la Covid-19 en pacientes con EB.

Clinical course of Covid-19 in a cohort of patients with Behçet disease

Objective: The implications of Covid-19 in patients with Behçet's disease (BD) are unknown. Patients with BD usually take long-term therapy with therapeutic agents that have been tested in Covid-19 patients. We aimed to assess the prevalence of Covid-19 in a cohort of patients with BD and investigate whether those patients with a long-term treatment with colchicine, tumor necrosis factor inhibitors (TNFi) or glucocorticoids are at reduced or increased prevalence of Covid-19 related clinical outcomes.MethodsA retrospective study was conducted among 244 patients with BD (86.1% females; mean age 43.95±11.11 years). Each participant completed an online questionnaire regarding demographics, medical conditions, dispensed colchicine, TNFi or oral glucocorticoids, Covid-19 infection, clinical symptoms and recovery.ResultsThe prevalence of Covid-19 infection was 14.75%. Regarding dose of colchicine, the presence of ageusia was lower in patients taking 0.5mg/day of colchicine compared to those taking 1.5mg/day (p=0.021). The prevalence of dyspnea was significantly higher in patients taking TNFi compared with those without therapy (p=0.032). With regards to oral glucocorticoids, no significant differences were found.ConclusionsThe prevalence of Covid-19 among patients with BD seems to be higher than that among the general population in Spain. Continuous TNFi therapy might increase the prevalence of worse clinical outcomes such as dyspnea; oral glucocorticoids and colchicine apparently provided no protection against the Covid-19 related clinical outcomes of patients with BD. (AU)

Objetivo: Se desconocen las implicaciones de la Covid-19 en pacientes con enfermedad de Behçet (EB). Los pacientes con EB generalmente tienen tratamiento de larga duración con agentes terapéuticos que se han probado en pacientes con Covid-19. Nuestro objetivo fue evaluar la prevalencia de la Covid-19 en una cohorte de pacientes con EB e investigar si los pacientes con un tratamiento de larga duración con colchicina, inhibidores del factor de necrosis tumoral (TNFi) o glucocorticoides tienen una prevalencia reducida o aumentada en los resultados clínicos de la Covid-19.MétodosSe realizó un estudio retrospectivo en 244 pacientes con EB (86,1% mujeres; edad media, 43,95±11,11 años). Cada participante completó un cuestionario en línea sobre datos demográficos, afecciones médicas, tratamiento con colchicina, TNFi o glucocorticoides orales, infección por Covid-19, síntomas clínicos y recuperación.ResultadosLa prevalencia de la infección por Covid-19 fue del 14,75%. En cuanto a la dosis de colchicina, la presencia de ageusia fue menor en los pacientes que tomaban 0,5mg/día de colchicina en comparación con los que tomaban 1,5mg/día (p=0,021). La prevalencia de disnea fue significativamente mayor en los pacientes que tomaban TNFi en comparación con aquellos sin terapia (p=0,032). Con respecto a los glucocorticoides orales, no se encontraron diferencias significativas.ConclusionesLa prevalencia de Covid-19 en pacientes con EB parece ser superior a la de la población general en España. La terapia continua con TNFi podría aumentar la prevalencia de peores resultados clínicos como la disnea; los glucocorticoides orales y la colchicina aparentemente no proporcionan protección contra los resultados clínicos relacionados con la Covid-19 en pacientes con EB. (AU)

The Relationship Between Health Literacy and Quality of Life, Attitudes and Perceptions of Covid-19 and Vaccination Among Patients with Systemic Autoimmune Diseases.

This study aimed to evaluate health literacy in patients (n=395) with systemic autoimmune diseases (SAD) and analyze their relationships with health-related quality of life (HRQoL), attitudes and beliefs about Covid-19 and vaccination, and perceptions of changes in medical care during the pandemic. This study was cross-sectional and the majority (81%) of particpants resided in Spain. An anonymous online survey was distributed to an online SAD association. Health literacy was measured using the European Health Literacy Survey Questionnaire (HLS-EU-Q16) and the SF-36 tool was used to assess HRQoL. More than half of patients (57.7%) have inadequate health literacy and the mean health literacy level was 9.63(5.66). Patients with inadequate health literacy levels presented the lowest HRQoL scores in all SF-36 domains (p < .001). Health literacy scores were positively correlated with all SF-36 domains (p < .001). The reservations to get vaccinated against Covid-19 were linked to health literacy level (p = 0.024). There are high levels of inadequate health literacy among patients with SAD and it is associated with worse HRQoL and risk attitudes about Covid-19 vaccination and medical care during the pandemic.

Dietary Sodium, Potassium, and Sodium to Potassium Ratio in Patients With Systemic Lupus Erythematosus.

Purpose: The aim of this study was to investigate the association between dietary sodium, potassium, and sodium:potassium ratio and clinical disease activity parameters, damage accrual, and cardiovascular disease risk factors in a population of patients with systemic lupus erythematous (SLE). Research design and study sample: A cross-sectional study including a total of 280 patients was conducted (90.4% females; mean age 46.9 ± 12.85 years). Data collection: The SLE Disease Activity Index (SLEDAI-2K) and the SDI Damage Index were used to assess disease activity and disease-related damage, respectively. A 24-hour diet recall was used to estimate dietary intake of sodium and potassium. Results: Dietary sodium intake was significantly associated with anti-dsDNA (ß = -.005; 95% CI [.002 .008]; p = .001) and complement C4 level (ß = -.002; 95% CI [-.003, .000]; p = .039). Dietary potassium intake was also significantly associated with complement C3 level (ß = -.004; 95% CI [-.007, -.001]; p = .021). Multiple logistic regression models revealed a positive association between dietary sodium intake and the risk of having hsCRP > 3 (p = .005) and an inverse association between dietary potassium intake and the risk of having hsCRP > 3 (p = .004). Conclusions: SLE patients with higher dietary sodium and lower dietary potassium intakes had an increased risk of higher hsCRP. Dietary sodium intake was significantly associated with anti-dsDNA and complement C4 level, while dietary potassium intake was associated with complement C3 level, supporting that dietary sodium and potassium intakes might play a key role in markers related to disease activity in SLE patients.

COVID-19 vaccine literacy in patients with systemic autoimmune diseases.

COVID-19 related infodemic is a threat to the successful COVID-19 vaccination campaigns. This might be especially apparent for patients with autoimmune diseases since there is no data available about the balance between benefits and risks of the newly developed COVID-19 vaccines in this population. We aim (i) to evaluate vaccine literacy skills in a population of patients with systemic autoimmune diseases, (ii) to examine the potential associations between vaccine literacy skills and sociodemographic characteristics and (iii) to analyze the relationships between attitudes, perceptions and beliefs about current vaccinations and vaccine literacy skills and sociodemographic characteristics. A cross-sectional study was conducted among 319 patients with systemic autoimmune diseases (92% females; 49.5% of patients in the 31-50 years age category). The vaccine literacy levels were determined using the Health Literacy about Vaccination in adulthood in Italian (HLVa-IT). Sociodemographic characteristics including gender, age, country and area of residence, civil status, socioeconomic status, educational attainment and occupational status were evaluated. The mean vaccine literacy functional and interactive-critical scores were 2.59 ± 0.74 and 3.07 ± 0.60, respectively. The vaccine literacy interactive-critical score was higher in females than in males (p = 0.048). Interactive-critical scores were associated with the area of residence, civil status and socioeconomic status, with the highest score in urban area of ≥ 100.000 inhabitants (p = 0.045), in widow patients (p = 0.023) and in patients with high socioeconomic status (p = 0.018). Significant differences were observed between the different education levels, for both the functional and the interactive-critical scores (p = 0.002 and p < 0.001, respectively), the highest score was observed in patients who completed a university degree. The level of vaccine literacy for functional and interactive-critical scales were medium. Area of residence, civil status and socioeconomic status represented determinants of vaccine literacy interactive-critical scale. Educational attainment also contributes to vaccine literacy functional scale. Insight into these factors is required to ensure an optimal vaccine literacy level in patients with autoimmune diseases. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12144-022-02713-y.

Clinical course of Covid-19 in a cohort of patients with Behçet disease.

OBJECTIVE: The implications of Covid-19 in patients with Behçet's disease (BD) are unknown. Patients with BD usually take long-term therapy with therapeutic agents that have been tested in Covid-19 patients. We aimed to assess the prevalence of Covid-19 in a cohort of patients with BD and investigate whether those patients with a long-term treatment with colchicine, tumor necrosis factor inhibitors (TNFi) or glucocorticoids are at reduced or increased prevalence of Covid-19 related clinical outcomes. METHODS: A retrospective study was conducted among 244 patients with BD (86.1% females; mean age 43.95±11.11 years). Each participant completed an online questionnaire regarding demographics, medical conditions, dispensed colchicine, TNFi or oral glucocorticoids, Covid-19 infection, clinical symptoms and recovery. RESULTS: The prevalence of Covid-19 infection was 14.75%. Regarding dose of colchicine, the presence of ageusia was lower in patients taking 0.5mg/day of colchicine compared to those taking 1.5mg/day (p=0.021). The prevalence of dyspnea was significantly higher in patients taking TNFi compared with those without therapy (p=0.032). With regards to oral glucocorticoids, no significant differences were found. CONCLUSIONS: The prevalence of Covid-19 among patients with BD seems to be higher than that among the general population in Spain. Continuous TNFi therapy might increase the prevalence of worse clinical outcomes such as dyspnea; oral glucocorticoids and colchicine apparently provided no protection against the Covid-19 related clinical outcomes of patients with BD.

Physical Fitness Attenuates the Impact of Higher Body Mass and Adiposity on Inflammation in Women With Systemic Lupus Erythematosus.

Aims: Higher body mass and adiposity represent independent contributors to the systemic low-grade inflammatory state often observed in patients with systemic lupus erythematosus (SLE). This study assessed the role of physical fitness in the association of body mass and adiposity with inflammation in women with SLE. Methods: A total of 77 women with SLE were included in this cross-sectional study. We obtained body mass index, waist-to-height ratio, and body fat percentage as indicators of body mass and adiposity. Inflammation was assessed through Serum levels of C-reactive protein, interleukin 6, and leptin. Cardiorespiratory fitness was assessed with the 6-minute walk test, range of motion with the back-scratch test, and muscular strength with handgrip dynamometry. Results: Cardiorespiratory fitness attenuated the association of both body mass index and body fat percentage with interleukin 6 (all, P<0.05). Range of motion attenuated the association of body mass index with interleukin 6 (P<0.05) and the association of body fat percentage with C-reactive protein (P<0.05). These interactions indicated that higher fitness was associated with a lower increase in inflammation per unit increase of body mass or adiposity. Muscular strength showed a non-significant trend to attenuate the association of body fat percentage with interleukin 6 (P=0.057) but potentiated the association of body fat percentage with leptin (P<0.05). Conclusion: These findings suggest that higher levels of cardiorespiratory fitness and range of motion might attenuate the impact of higher body mass and adiposity on inflammation in women with SLE. The role of muscular strength requires further investigation.

Sensorimotor tongue evaluation and rehabilitation in patients with sleep-disordered breathing: a novel approach.

STUDY OBJECTIVES: To evaluate tone, apraxia and stereognosis dysfunctions in patients with SDB compared with healthy controls, and to monitor the effectiveness of Airway Gym® as an easy-to-use myofunctional therapy (MT) modality in terms of the tongue's motor and sensory responses, comparing results before and after therapy. METHODS: This was a prospective, non-randomised pilot study of 25 patients with moderate to severe obstructive sleep apnoea-hypopnoea syndrome (OSAHS), 25 patients with primary snoring (PS) and 20 healthy controls. Qualitative and quantitative instruments-Iowa Oral Performance Instrument (IOPI), lingual apraxia and stereognosis tests were used to assess tongue sensorimotor function. RESULTS: 22 patients with PS, 21 with OSAHS and all 20 controls ended the therapy. In OSAHS, the Epworth Sleepiness Scale score decreased from 16 ± 7.3 to 12 ± 4.5 after therapy (p = 0.53). In PS and OSAHS groups, the IOPI scores increased significantly. These measures did not change significantly in the controls. Lingual apraxia testing showed that controls performed all the manoeuvres, whereas PS 5.6 ± 1.4 and OSAHS 4.5 ± 1.9 (p = 0.14). In the stereognosis test, the mean number of figures recognised was 2.6 ± 2.2 in OSAHS, 3.3±1.2 in PS and 5.7±0.9 in control group (p < 0.05). Patients with OSAHS recognised circles and ovals less often. CONCLUSION: Using the Airway Gym® app produced improvements in sensorimotor tongue function in patients with SDB, due to continuous stimulation of the brain based on proprioceptive training required to localise responses when doing the exercises.

Clinical and serological associations of autoantibodies in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by the formation of antigen-antibody complexes which trigger an immune response. We investigate certain autoantibodies including nucleosome, double-stranded DNA (dsDNA), Smith, ribonucleoprotein, and Sjögren's syndrome-related antigens, and examine their associations with disease activity, damage accrual, and SLE-related clinical and serological manifestations in patients with SLE. We conducted a cross-sectional study with a total 293 patients (90.4% female, mean age 46.87±12.94 years) and used the Systemic Lupus Erythematosus Disease Activity Index 2000 and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) to evaluate disease activity and disease-related damage, respectively. Systemic Lupus Erythematosus Disease Activity Index scores were significantly higher in anti-nucleosome-positive (3.87±2.72 vs 2.52±2.76, p=0.004) and anti-dsDNA-positive (3.08±2.91 vs 2.04±2.48, p=0.010) patients compared with patients without these antibodies. SDI scores were also significantly higher in anti-nucleosome-positive patients (1.61±1.99 vs 0.89±1.06, p=0.004). The presence of antinucleosome (p=0.019) and anti-dsDNA antibodies (p=0.001) both correlated significantly with the incidence of nephritis; anti-La antibodies were associated with arthritis (p=0.022), and we also observed a relationship between the presence of antinucleosome antibodies and leukopenia (p=0.011). Patients with antinucleosome or anti-dsDNA antibodies had a higher disease activity and were likely to have nephritis. Antinucleosome was also associated with more damage accrual. A greater understanding of these autoantibodies could lead to the development of new approaches to more accurate assessments of SLE.