Detection of systemic autoimmune diseases in an ongoing assessment program for hand arthralgias. A comparative analysis with inflammatory and non-inflammatory arthropathies.

INTRODUCTION: Arthralgias are prevalent in systemic autoimmune rheumatic diseases (SARD), emphasizing the need for early recognition. This study aimed to estimate SARD frequency and compare clinical, laboratory, and imaging findings among SARD, non-inflammatory arthralgia (NIA), and RA in patients with hand arthralgias. METHODS: A prospective evaluation program included individuals aged ≥18 with hand arthralgias. Baseline assessments covered clinical, laboratory, ultrasound, and radiography. Follow-up diagnoses categorized patients into SARD, NIA, and RA groups. Comparison between groups was performed using parametric and non-parametric tests. Two multivariate logistic regression analyzes were performed using the final diagnosis of SARD as the dependent variable (NIA and RA). ROC curves were calculated in those variables that presented an independent association in the multivariate analysis. RESULTS: Among 1053 patients, 9.6% were SARD (SLE 47%). Comparing SARD with NIA revealed higher CRP levels, power Doppler, less rhizarthrosis in ultrasound, and more ANA positivity in SARD patients. Distinct differences were observed between SARD and RA patients in terms of pain levels, swollen joints, metacarpophalangeal involvement and morning symptoms. Diagnostic markers demonstrated specific sensitivities and specificities: ANA for SARD versus NIA (82%, 34%), US not finding rhizarthrosis for SARD versus NIA (66%, 85%), CRP (cut-off >2.5 mg/L) sensitivity 52%, specificity 60%, AUC 0.62, RA antibodies (RF, 11 IU/mL) sensitivity 76%, specificity 74%, AUC 0.8, ACPA (1.25) sensitivity 50%, specificity 98%, AUC 0.7, ANA+ sensitivity 95%, specificity 32%, AUC 0.7, and US absence of synovitis sensitivity 82%, specificity 34%, AUC 0.75. CONCLUSION: This study highlights distinct clinical, laboratory, and imaging features differentiating SARD-related hand arthralgia from non-SARD hand arthralgia and RA.

Ocular cicatricial pemphigoid: is there an association with autoimmune diseases?

PURPOSE: To assess the prevalence of autoimmune diseases (ADs) associated with ocular cicatricial pemphigoid (OCP) and analyze clinical, laboratory, and treatment associations between these entities. METHODS: A multicentre cross-sectional study of patients with an OCP diagnosis. The population was divided into two groups according to their association with other ADs or not. Clinical, laboratory and treatment variables were described and compared between groups. A multivariable logistic regression analysis was performed to identify variables that could suggest the association between OCP and ADs. RESULTS: Eighty-eight patients were recruited, with a mean age at diagnosis of 64.3 years (SD 11.9). Biopsy was performed in 86.8% of the patients. There was a median delay of 2 years from the onset of symptoms to diagnosis. Extraocular involvement was evidenced in 11.5%. The group associated with ADs included 24 patients (27.3%). The most prevalent diagnosis was Sjögren´s syndrome. Hypergammaglobulinemia was associated with ADs and OCP, adjusted for age, sex, smoking, skin and mucosal involvement, and erythrocyte sedimentation rate (OR 8.7; 95%CI 1.6-46.8; p = 0.012). CONCLUSIONS: Due to OCP's autoimmune nature, it could coexist with other ADs. This study observed that more than a quarter of the population presented with this association, and hypergammaglobulinemia could suggest it.

Chronic neutropenia in childhood: experience of a tertiary center.

OBJECTIVE: To assess the prevalence of chronic neutropenia (CN) and the clinical profile of patients with CN aged up to 18 years, followed in the pediatric hematology, rheumatology, or immunology outpatient clinic of a tertiary medical center from May 1, 2018, to 30 April 2019. METHODS: Retrospective observational study carried out by collecting data from the patient's medical charts. CN was defined as absolute neutrophil count (ANC) below 1.5 × 109/L lasting over three months. Autoimmune neutropenia (AIN) was defined by clinical criteria and an over twofold increase in ANC after glucocorticoid stimulation. AIN was considered secondary when associated with autoimmune or immunoregulatory disorders. Wilcoxon and Fisher's exact tests were used to compare variables; the significance level was 5 %. RESULTS: A total of 1,039 patients were evaluated; 217 (20 %) presented CN. Twenty-one (2 %) had AIN, classified as primary in 57 % of the cases. The average age at the onset of symptoms was 38.6 months. During follow-up, patients had 4.2 infections on average; frequency was higher among patients with secondary AIN (p = 003). Isolated neutropenia occurred in 43 % of the patients with AIN. Neutropenia resolved in eight (38 %) of the 21 patients with AIN within 19.6 months on average. Eight patients with secondary AIN met the criteria for Inborn Errors of Immunity. CONCLUSION: AIN prevalence was 2 %. Most cases were first evaluated by a pediatric immunologist or rheumatologist rather than a pediatric hematologist. This study highlights the need for a multidisciplinary approach involving a pediatric immunologist, rheumatologist, and hematologist.

Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies.

INTRODUCTION: Overlap autoimmune syndromes (OAS) and mixed connective tissue disease (MCTD) are rare in children. We performed a retrospective, longitudinal and descriptive study of Afro-Caribbean patients from the French West Indies followed for MCTD and OAS to describe their characteristics and outcomes during childhood. METHODS: Retrospective study from January 2000 to 2023. Listings of patients were obtained from multiple sources: computerized hospital archives and national hospital-based surveillance system, registry of pediatricians and adult specialists in internal medicine and the national registry for rare diseases. MCTD was defined according to Kasukawa's criteria. OAS was defined as overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and dermatomyositis/autoimmune myositis (DM/AM). RESULTS: Sixteen patients were included over a 23-year period (10 MCTD and 6 OAS). The incidence was 0.23 per 100,000 children-years. The mean age at diagnosis was 11.9 years old (2.4-17) with median follow up of 7.9 years (2.1-19.6). SLE phenotype was present in the highest, followed by SSc and DM/AM. Patients had an average of three flares during childhood (1-7). A quarter (25%) had symptomatic pulmonary arterial hypertension (PAH). Ninety-four percent received steroids during follow-up and 88% required a corticosteroid-sparing therapy. Three patients (19%) developed SLE after more than 10y of follow-up. There were no death and no chronic organ failure. CONCLUSION: This is the largest pediatric cohort of MCTD and OAS in Afro-descendant patients treated in a country with a high standard of care. The clinical evolution did not differ between MCTD and OAS. The main complication was PAH, more frequent in our cohort.

El reto del internista ante las enfermedades autoinmune reumáticas / The Challenge Faced by Internists in Rheumatological Autoimmune Diseases

El progreso científico de un continente o de un país en específico puede medirse a través de los estudios bibliométricos que tienen como objeto el tratamiento y el análisis cuantitativo de las publicaciones científicas. Este se basa en el número de publicaciones, autores, citas bibliográficas y áreas de investigaciones comunes.1 En Latinoamérica estos datos sobre las enfermedades autoinmunes (EA) resultan poco conocidos. Entre los años de 2007 a 2017 se encontraron 7184 publicaciones sobre este tema, y es el lupus eritematoso sistémico (LES) el tópico más publicado seguido de las enfermedades inflamatoria intestinal y artritis reumatoide (AR). Cuba precedida por Perú ocupó el octavo lugar. Estas cifras resultan bajas en relación con líderes como Brasil, México y Argentina, aunque se considera meritorio que Cuba esté entre los 10 primeros países entre 24 naciones del continente.2 No obstante, al evaluar cuanto se ha logrado, nos enfrentamos al reto de profundizar aún más en el conocimiento sobre la epidemiología, los complejos mecanismos patogénicos y las disímiles formas clínicas de expresión de estas entidades, para continuar el avance en las dianas terapéuticas. Los estudios epidemiológicos comunitarios para el control de la prevalencia de las enfermedades reumáticas y la discapacidad asociada están bajo la iniciativa de la Organización Mundial de la Salud y la Liga Internacional de Reumatología (COPCORD/WHO/ILAR), que se realiza en Cuba, publicados en (Journal Clinical of Rheumatology) en el 2009; esto ha permitido establecer la prevalencia de la AR en el 1,2 por ciento y el lupus en 60/100,000 habitantes.3 Los datos citados por la comunidad internacional han sentado las bases para subsiguientes estudios de investigación, para así propiciar a los directivos de la salud tomas de decisiones en cuanto a las necesidades de los recursos materiales y humanos. La AR es una enfermedad inflamatoria crónica, caracterizada por la presencia de autoanticuerpos causales de una cascada de citoquinas proinflamatorias determinantes en el daño articular irreversible. El tratamiento se ha basado en el uso de drogas convencionales, como el metotrexato, leflunomida, azulfidina y sus combinaciones, las cuales ofrecen algunos beneficios pero sus efectos están limitados por la toxicidad.4 El uso de drogas biológicas para la AR, como los anticuerpos anti-TNF-α, el tocilizumab que va dirigido contra el receptor de IL-6, abatacept que previene la coestimulación, el rituximab Ac, anticélulas B CD20 y el tofacitinib como un nuevo inhibidor de las Janus quinasa (JAK, por sus siglas en inglés) de reciente aprobación por las agencias regulatorias de Estados Unidos (FDA) y la agencia europea (EMA) han mejorado sustancialmente la eficacia terapéutica antes alcanzada.5 Las experiencias de los reumatólogos cubanos fueron recogidas en el documento Nacional de Consenso realizado en nuestro país sobre tratamiento de la AR, a partir de la consideración del documento de postura latinoamericano discutido en la localidad chilena de Reñaca con la presencia de eminentes reumatólogos cubanos.6 La atención en la práctica diaria de los pacientes con LES en la fase de la actividad severa y con daño acumulado, presupone un desafío para los especialistas. Se trata de una enfermedad poligénica asociada a la producción de autoanticuerpos, con pérdida de la autotolerancia, el desarrollo de una respuesta inmune alterada y la expresión de diversas manifestaciones clínicas que difícultan establecer un diagnóstico temprano, aunque, cuenta con criterios de clasificación y diagnóstico por La Liga Europea y el Colegio Americano de Reumatología (ACR).7) Además, puede remedar afecciones como la tuberculosis y la infección por el virus de la inmunodeficiencia humana (VIH/SIDA). Esta nueva terapia de antirretrovirales ha sido muy efectiva, por lo tanto ha aumentado la supervivencia de los pacientes con el (VIH), pero a partir de la emergencia sobre los nuevos desórdenes autoinmunes reumáticos se convierte para los especialistas en un nuevo reto clínico.8,9 Cuba está entre los 21 países fundadores del Grupo Latinoamericano para el estudio del LES (GLADEL), con la participación y coautoría de múltiples estudios y publicaciones internacionales de impacto.10,11,12 Consideramos una fortaleza que los especialistas cubanos como Marlene Guibert y Gil Reyes hayan logrado la inclusión de una serie proporcional de pacientes en las cohortes internacionales de pacientes con AR, con lupus y que han asumido el reto de la coautoría de las primeras Guías Latinoamericanas de Tratamiento acerca del lupus eritematoso sistémico.13 En el caso de la ES es una rara enfermedad del tejido conectivo en la cual intervienen mediadores profibróticos como el factor vascular endotelial de crecimiento (VEGF), el factor 4 de activación de plaquetas (PF4), y el sistema inmune innato con inflamación, vasculopatía y fibrosis, incluso intersticial pulmonar en 35-52 por ciento de los casos y la mortalidad con el 20-40 por ciento14,15 En general resultan escasos los estudios publicados en el país. En la Revista Cubana de Reumatología aparecen 2 estudios epidemiológicos de nuestra autoría: uno publicado en el año 2007 y el otro en el año 2014, ambos artículos mostraron una evaluación clínico-epidemiológica de una serie de 34 pacientes en dos centros de referencia nacional, que describen las manifestaciones gastrointestinales severas en pacientes con ES realizadas en el hospital CIMEQ. Como limitaciones señalamos que el estudio realizado fue descriptivo y desarrollado en una pequeña serie.16Pérez y otros,17 en el hospital Hermanos Ameijeiras, mostraron los resultados satisfactorios del tratamiento en 2 grupos de pacientes con ES, enfermedad pulmonar......(AU)

Actividad y discapacidad en pacientes cubanos con artritis reumatoide como factores que influyen en la sexualidad / Activity and Disability in Cuban Patients with Rheumatoid Arthritis as Factors that Influence Sexuality

Introducción: La artritis reumatoide es una enfermedad autoinmune de carácter inflamatorio y crónico. La afectación en la esfera sexual es frecuente, compromete a ambos sexos y se relaciona con factores como el dolor, la discapacidad y el consumo de medicamentos. Esta afectación no ha sido suficientemente abordada en la literatura a pesar de su prevalencia, y en Cuba no se han reportado hasta el momento estudios relacionados sobre este tema de investigación. Objetivo: Determinar el impacto de la artritis reumatoide en la sexualidad y su relación en la actividad y la discapacidad. Métodos: Se realizó un estudio monocéntrico, transversal, descriptivo. Se incluyeron los pacientes con un diagnóstico de artritis reumatoide en el período comprendido de septiembre de 2019 a junio de 2021. Se utilizó el cuestionario Qualisex para evaluar el impacto de la artritis reumatoide en la sexualidad. Resultados: En el estudio doscientos veintiséis pacientes fueron incluidos, la media de edad fue de 53,38 años (DE ± 12,22) el 82,7 por ciento fueron mujeres. Al responder el autocuestionario Qualisex el 73,9 por ciento de los sujetos presentaron afectación en la sexualidad. No se estableció una relación significativa entre la afectación en la esfera sexual y el tiempo de evolución. A diferencia de los niveles altos de actividad y discapacidad. Conclusiones: En la población estudiada se presentó afectación en la sexualidad, no obstante, esta no se relacionó con el tiempo de evolución de la artritis reumatoide. Se encontró asociación entre la actividad de la enfermedad y la capacidad funcional con la afectación en la esfera sexual(AU)

Introduction: Rheumatoid arthritis is a chronic, inflammatory autoimmune disease. Disorders in the sexual sphere is frequent, it affects both sexes and it is related to factors such as pain, disability and medication consumption. This condition has not been sufficiently addressed in the literature despite its prevalence and in Cuba no studies related to the topic under study have been reported to date. Objective: To determine the impact of rheumatoid arthritis on sexuality and its relationship with activity and disability. Methods: A monocentric, cross-sectional and descriptive study was carried out on patients with a diagnosis of rheumatoid arthritis, from September 2019 to June 2021. The Qualisex questionnaire was used to evaluate the impact of rheumatoid arthritis on sexuality. Results: Two hundred twenty-six patients were included, the mean age was 53.38 years (SD ± 12.22) and 82.7percent were women. When answering the Qualisex self-questionnaire, 73.9percent of the subjects had effects in their sexuality. No significant relationship was established between the involvement in the sexual sphere and the time of evolution. Conclusions: The impact on sexuality in the studied population was not related to the duration of rheumatoid arthritis. On the other hand, an association was found between disease activity and functional capacity with effects in the sexual sphere(AU)

Clinical characteristics and outcomes of patients with inflammatory and autoimmune rheumatological diseases admitted for intensive care in Colombia.

OBJECTIVE: Contemporary studies reporting outcomes of critical care in patients with inflammatory and autoimmune rheumatological diseases are scarce. This study describes 15 years of experience from 2005-2019 in a Colombian referral hospital. METHODS: This observational, descriptive, consecutive case series study was performed on adult patients with inflammatory and autoimmune rheumatic diseases who were admitted to the intensive care unit (ICU) of the San Ignacio University Hospital in Bogotá (Colombia), from January 1, 2005, to December 21, 2019. We describe the sociodemographic characteristics, admission causes and criteria, lengths of stay, immunosuppressive treatment, systemic support, and mortality. RESULTS: The study included 300 patients with a median age of 48 years [interquartile range (IQR) 31-62 years], predominantly female (76%). Disease exacerbations (30%), infections (17.6%), and cardiovascular diseases (15%) were the main causes of admission. Respiratory failure (23%) most commonly caused by septic shock (24%) was the principal indication for intensive care admission. The most frequent infections were community-acquired pneumonia (11.6%) and soft-tissue infections (9%). In 40.3% of patients, inotropic and vasopressor support was required. The median length of stay was 4 days (IQR 2-8), and global mortality was 21.6%. CONCLUSIONS: Rheumatic diseases in the ICU are still associated with high morbidity and mortality. Patients with inflammatory and autoimmune rheumatic diseases require a meticulous clinical approach, strict clinical monitoring, frequent assessment of complications, evaluation of systemic support needs, and specific management.

Association of vitiligo with multiple cutaneous and extra-cutaneous autoimmune diseases: a nationwide cross-sectional study.

Previous studies found conflicting results about associations of vitiligo with different autoimmune diseases. To evaluate associations of vitiligo with multiple autoimmune diseases. A cross-sectional study representative of 612,084,148 US patients from the Nationwide Emergency Department Sample (NEDS) 2015-2019 was performed. Vitiligo and autoimmune diseases were identified using International Classification of Diseases-10 codes. The most frequent autoimmune disorders in patients with vitiligo were type 1 diabetes, rheumatoid arthritis, systemic lupus erythematosus (SLE), autoimmune thyroiditis, Addison's disease, and systemic sclerosis (SSc). Vitiligo was associated with any autoimmune disorder (adjusted odds ratio [95% confidence interval] 1.45 [1.32-1.58]). Cutaneous disorders with largest effect-sizes were alopecia areata (186.22 [115.31-300.72]) and SSc (32.13 [25.28-40.82]). Non-cutaneous comorbidities with largest effect-sizes were primary sclerosing cholangitis (43.12 [18.98-97.99]), pernicious anemia (41.26 [31.66-53.78]), Addison's disease (33.85 [26.68-42.9]), and autoimmune thyroiditis (31.65 [26.34-38.02]). Vitiligo is associated with multiple cutaneous and non-cutaneous autoimmune diseases, especially in females and older age.

Post-acute COVID-19 in three doses vaccinated autoimmune rheumatic diseases patients: frequency and pattern of this condition.

BACKGROUND: Data on post-acute COVID-19 in autoimmune rheumatic diseases (ARD) are scarce, focusing on a single disease, with variable definitions of this condition and time of vaccination. The aim of this study was to evaluate the frequency and pattern of post-acute COVID-19 in vaccinated patients with ARD using established diagnosis criteria. METHODS: Retrospective evaluation of a prospective cohort of 108 ARD patients and 32 non-ARD controls, diagnosed with SARS-CoV-2 infection (RT-PCR/antigen test) after the third dose of the CoronaVac vaccine. Post-acute COVID-19 (≥ 4 weeks and > 12 weeks of SARS-CoV-2 symptoms) were registered according to the established international criteria. RESULTS: ARD patients and non-ARD controls, balanced for age and sex, had high and comparable frequencies of ≥ 4 weeks post-acute COVID-19 (58.3% vs. 53.1%, p = 0.6854) and > 12 weeks post-acute COVID-19 (39.8% vs. 46.9%, p = 0.5419). Regarding ≥ 4 weeks post-acute COVID-19, frequencies of ≥ 3 symptoms were similar in ARD and non-ARD controls (54% vs. 41.2%, p = 0.7886), and this was also similar in > 12 weeks post-acute COVID-19 (68.3% vs. 88.2%, p = 0.1322). Further analysis of the risk factors for ≥ 4 weeks post-acute COVID-19 in ARD patients revealed that age, sex, clinical severity of COVID-19, reinfection, and autoimmune diseases were not associated with this condition (p > 0.05). The clinical manifestations of post-acute COVID-19 were similar in both groups (p > 0.05), with fatigue and memory loss being the most frequent manifestations. CONCLUSION: We provide novel data demonstrating that immune/inflammatory ARD disturbances after third dose vaccination do not seem to be a major determinant of post-acute COVID-19 since its pattern is very similar to that of the general population. Clinical Trials platform (NCT04754698).

[Prevalence of persistent hypogammaglobulinemia in patients with autoimmune rheumatic disease who received treatment with Rituximab in a national medical center in Mexico]. / Prevalencia de hipogammaglobulinemia persistente en pacientes con enfermedad autoinmune que reciben rituximab en un centro de referencia nacional en México.

OBJECTIVE: To describe the prevalence of persistent hypogammaglobulinemia in patients receiving Rituximab as a treatment for autoimmune rheumatological diseases. METHODS: A transversal, retrospective and unicentric study, carried out in patients with autoimmune rheumatic diseases who were admitted to the Rheumatology service of the Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, Mexico City, to receive treatment with rituximab between January 2013 and January 2018. Descriptive and inferential statistics of serum levels of immunoglobulins, clinical-demographic characteristics, diagnosis, and treatment received were performed. RESULTS: from 262 patients with autoimmune rheumatological disease who received treatment with Rituximab; We identified 8 patients with persistent hypogammaglobulinemia (6 women and 2 men), this is a prevalence of 3.1%. No associated factors with the development of hypogammaglobulinemia were identified. CONCLUSIONS: Until now, no associated prognostic or predictive factors have been identified with persistent hypogammaglobulinemia. Additional prospective studies are required to understand more precisely the implications of persistent hypogammaglobulinemia in patients with autoimmune diseases.

OBJECTIVO: Determinar la prevalencia de hipogammaglobulinemia persistente en pacientes con enfermedades reumatológicas autoinmunes que reciben rituximab. MÉTODOS: Estudio trasversal, retrospectivo y unicéntrico, emprendido en pacientes con enfermedades reumatológicas autoinmunes, que acudieron a la Consulta externa del servicio de Reumatología del Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, Ciudad de México, entre enero de 2013 y enero de 2018, para recibir tratamiento con rituximab. El análisis de los datos se efectuó con estadística descriptiva e inferencial, para la evaluación de las concentraciones séricas de inmunoglobulinas, características clínico demográficas, diagnóstico y tratamiento. RESULTADOS: Estudio trasversal, retrospectivo y unicéntrico, emprendido en pacientes con enfermedades reumatológicas autoinmunes, que acudieron a la Consulta externa del servicio de Reumatología del Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, Ciudad de México, entre enero de 2013 y enero de 2018, para recibir tratamiento con rituximab. El análisis de los datos se efectuó con estadística descriptiva e inferencial, para la evaluación de las concentraciones séricas de inmunoglobulinas, características clínico demográficas, diagnóstico y tratamiento. CONCLUSIONES: Hasta el momento no se han identificado factores asociados, pronósticos o predictivos, con hipogammaglobulinemia persistente. Se requieren estudios prospectivos adicionales para conocer con mayor precisión las implicaciones de la hipogammaglobulinemia persistente en pacientes con enfermedades autoinmunes.

Pain in individuals with idiopathic inflammatory myopathies, other systemic autoimmune rheumatic diseases, and without rheumatic diseases: A report from the COVAD study.

OBJECTIVES: To compare pain intensity among individuals with idiopathic inflammatory myopathies (IIMs), other systemic autoimmune rheumatic diseases (AIRDs), and without rheumatic disease (wAIDs). METHODS: Data were collected from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) study, an international cross-sectional online survey, from December 2020 to August 2021. Pain experienced in the preceding week was assessed using numeral rating scale (NRS). We performed a negative binomial regression analysis to assess pain in IIMs subtypes and whether demographics, disease activity, general health status, and physical function had an impact on pain scores. RESULTS: Of 6988 participants included, 15.1% had IIMs, 27.9% had other AIRDs, and 57.0% were wAIDs. The median pain NRS in patients with IIMs, other AIRDs, and wAIDs were 2.0 (interquartile range [IQR] = 1.0-5.0), 3.0 (IQR = 1.0-6.0), and 1.0 (IQR = 0-2.0), respectively (P < 0.001). Regression analysis adjusted for gender, age, and ethnicity revealed that overlap myositis and antisynthetase syndrome had the highest pain (NRS = 4.0, 95% CI = 3.5-4.5, and NRS = 3.6, 95% CI = 3.1-4.1, respectively). An additional association between pain and poor functional status was observed in all groups. Female gender was associated with higher pain scores in almost all scenarios. Increasing age was associated with higher pain NRS scores in some scenarios of disease activity, and Asian and Hispanic ethnicities had reduced pain scores in some functional status scenarios. CONCLUSION: Patients with IIMs reported higher pain levels than wAIDs, but less than patients with other AIRDs. Pain is a disabling manifestation of IIMs and is associated with a poor functional status.

Cardiovascular health worsening in patients with autoimmune rheumatological diseases during the COVID-19 pandemic.

Rheumatic autoimmune diseases are associated with a myriad of comorbidities. Of particular importance due to their morbimortality are cardiovascular diseases. COVID-19 greatly impacted the world population in many different areas. Patients with rheumatic diseases had to face changes in their healthcare, in addition to unemployment, a decrease in physical activity, social isolation, and lack of access to certain medications. This review summarizes the impact of COVID-19 pandemic on cardiovascular risk factors, comorbidities, and unhealthy behaviors in patients with rheumatic inflammatory autoimmune diseases, particularly focused on rheumatoid arthritis and systemic lupus erythematosus. Searches were carried out in MEDLINE/PubMed and Scopus from August to December 2022. Four reviewers screened the title and abstract of retrieved records. Potentially eligible reports were then reviewed in full text. Differences were reconciled by either consensus or discussion with an external reviewer. During the COVID-19 pandemic, patients with rheumatic diseases showed an increase in the prevalence of mental health disorders (43.2-57.7%), reduced physical activity (56.8%), and a worsening in eating behaviors. Alcohol intake increased (18.2%), especially in early phases of the pandemic. Smoking prevalence decreased (28.2%). Dyslipidemia and hypertension showed no changes. The pandemic and lockdown affected rheumatic patients not only in disease-related characteristics but in the prevalence of their cardiovascular comorbidities and risk factors. Lifestyle changes, such as healthy eating, physical activity, and optimal management of their rheumatic diseases and comorbidities, are essential to manage the long-lasting consequences of the COVID-19 outbreak. Key Points • During the COVID-19 pandemic, anxiety, depression, sedentarism, obesity, and a worsening in eating behaviors increased. •Patients with rheumatic diseases and comorbidities have worse clinical outcomes and a higher cardiovascular disease burden than those without them. •Comparative studies are necessary to precisely elucidate the pandemic's impact on the prevalence of cardiovascular disease, risk factors, and comorbidities in patients with rheumatoid arthritis and systemic lupus erythematosus.

COVID-19 severity and vaccine breakthrough infections in idiopathic inflammatory myopathies, other systemic autoimmune and inflammatory diseases, and healthy controls: a multicenter cross-sectional study from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) survey.

OBJECTIVES: We aimed to compare the spectrum and severity of COVID-19 and vaccine breakthrough infections (BIs) among patients with IIMs, other systemic autoimmune and inflammatory diseases (SAIDs), and healthy controls (HCs). METHODS: This is a cross-sectional study with data from the COVAD study, a self-reported online global survey that collected demographics, COVID-19 history, and vaccination details from April to September 2021. Adult patients with at least one COVID-19 vaccine dose were included. BIs were defined as infections occurring > 2 weeks after any dose of vaccine. Characteristics associated with BI were analyzed with a multivariate regression analysis. RESULTS: Among 10,900 respondents [42 (30-55) years, 74%-females, 45%-Caucasians] HCs were (47%), SAIDs (42%) and IIMs (11%). Patients with IIMs reported fewer COVID-19 cases before vaccination (6.2%-IIM vs 10.5%-SAIDs vs 14.6%-HC; OR = 0.6, 95% CI 0.4-0.8, and OR = 0.3, 95% CI 0.2-0.5, respectively). BIs were uncommon (1.4%-IIM; 1.9%-SAIDs; 3.2%-HC) and occurred in 17 IIM patients, 13 of whom were on immunosuppressants, and 3(18%) required hospitalization. All-cause hospitalization was higher in patients with IIM compared to HCs [23 (30%) vs 59 (8%), OR = 2.5, 95% CI 1.2-5.1 before vaccination, and 3 (18%) vs 9 (5%), OR = 2.6, 95% CI 1.3-5.3 in BI]. In a multivariate regression analysis, age 30-60 years was associated with a lower odds of BI (OR = 0.7, 95% CI 0.5-1.0), while the use of immunosuppressants had a higher odds of BI (OR = 1.6, 95% CI 1.1-2.7). CONCLUSIONS: Patients with IIMs reported fewer COVID-19 cases than HCs and other SAIDs, but had higher odds of all-cause hospitalization from COVID-19 than HCs. BIs were associated with the use of immunosuppressants and were uncommon in IIMs.

Combined Ophthalmology and Rheumatology Evaluation in Systemic Autoimmune Diseases with Ocular Involvement: A Third Level Reference Center in Mexico.

AIM: A cross-sectional descriptive study to determine the frequency of ocular manifestations associated with systemic autoimmune diseases in a third-level hospital in Mexico. METHODS: Records from 2014 to 2017 at the Inflammatory Eye Disease Clinic of the Asociación Para Evitar la Cegueraen México were examined by both an ophthalmologist and a rheumatologist on the same day. Diagnosis was achieved from initial ocular manifestations with later systemic assessment. RESULTS: Out of 311 medical records, 276 were included, 75% of the patients were female. Keratoconjunctivitis sicca was the most frequent ocular manifestation (33.3%), followed by anterior uveitis (29.5%), scleritis (23.2%), and peripheral ulcerative keratitis (7.2%). The leading autoimmune diseases were spondyloarthritis (29%), rheumatoid arthritis (28.6%), primary Sjögren's syndrome (10.5%) and granulomatosis with polyangiitis (9.1%). 41.3% of systemic disease diagnoses were made after an initial ocular manifestation. CONCLUSIONS: Inflammatory eye manifestations can imply systemic autoimmune diseases. It is crucial to suspect and confirm this association and provide timely interdisciplinary management.

Clinical patterns and risk factors in scleritis: a multicentric study in Colombia.

PURPOSE: This study aims to describe the clinical characteristics of scleritis in a large cohort of Colombian patients and identify factors associated with the clinical presentation. METHODS: Retrospective case series of patients with scleritis from 2015 to 2020. Clinical records were obtained from seven uveitis referral centers in Colombia. Patients with a diagnosis of episcleritis were excluded. RESULTS: We evaluated 389 patients with scleritis (509 eyes). There was a female predominance (75.6%) with a mean age of 51 ± 15 years. Most cases were noninfectious (94.8%) and unilateral (69.2%). The most frequent type of inflammation was diffuse anterior scleritis (41.7%), followed by nodular scleritis (31.9%) and necrotizing scleritis (12.3%). Systemic autoimmune diseases were found in 41.3% of patients, the most common being rheumatoid arthritis (18.5%) and granulomatosis with polyangiitis (5.9%). Polyautoimmunity was found in 10.4% of those with a systemic autoimmune disease. The most frequent treatment was systemic steroids (50.9%), followed by systemic NSAIDs (32.4%). Steroid-sparing immunosuppression was required in 49.1% of patients. Systemic autoimmune diseases were more common in patients with necrotizing scleritis and those older than 40 years of age. Best-corrected visual acuity of 20/80 or worse at presentation was more common in necrotizing scleritis and subjects with associated uveitis, ocular hypertension, or who were over 40 years of age. CONCLUSIONS: This is the first study in Colombia and the largest in Latin America describing the clinical characteristics and presentation patterns of scleritis. The most common presentation was in females, with unilateral, anterior diffuse noninfectious scleritis. Systemic autoimmune diseases and polyautoimmunity were frequent, as was the need for steroid-sparing immunosuppression. Age over 40 and necrotizing scleritis were associated with higher odds of having a systemic autoimmune disease and worse visual acuity at presentation.

Is post-COVID syndrome an autoimmune disease?

INTRODUCTION: Post-COVID syndrome (PCS) is recognized as a new entity in the context of SARS-CoV-2 infection. Though its pathogenesis is not completely understood, persistent inflammation from acute illness and the development of autoimmunity play a critical role in its development. AREAS COVERED: The mechanisms involved in the emergence of PCS, their similarities with post-viral and post-care syndromes, its inclusion in the spectrum of autoimmunity and possible targets for its treatment. EXPERT OPINION: An autoimmune phenomenon plays a major role in most causative theories explaining PCS. There is a need for both PCS definition and classification criteria (including severity scores). Longitudinal and controlled studies are necessary to better understand this new entity, and to find what additional factors participate into its development. With the high prevalence of COVID-19 cases worldwide, together with the current evidence on latent autoimmunity in PCS, we may observe an increase of autoimmune diseases (ADs) in the coming years. Vaccination's effect on the development of PCS and ADs will also receive attention in the future. Health and social care services need to develop a new framework to deal with PCS.

Primary Sjögren syndrome and development of another autoimmune rheumatic disease during the follow-up.

BACKGROUND: Primary Sjögren syndrome (pSS) is a chronic autoimmune disease with its main target being exocrine glands, and is the connective tissue disease more frequently associated with other autoimmune diseases. The aim of this study was to assess the frequency of another autoimmune rheumatic disease (ARD) developed in primary Sjögren syndrome (pSS) patients and to describe it's clinical, serological and histologic characteristics. MATERIALS AND METHODS: This is a retrospective cohort study. Data of patients with pSS diagnosis (American-European criteria 2002), included in the GESSAR database (Grupo de Estudio Síndrome de Sjögren, Sociedad Argentina de Reumatología) were analyzed. The development of a second ARD was registered during the follow up. RESULTS: 681 patients were included, 94.8% female. The mean age was 54 (SD 14) years and mean age at diagnosis of 50 (SD 13) years. The mean follow-up was 4.7 (SD 4.9) years; 30 patients (4.41%, CI 95%: 3.1-5.7) developed a second ARD during the follow up, incidence rate was 9.1/1000 patients-year (IR 95%: 5.8-12.4/1000 patients-year), the most frequent being rheumatoid arthritis (RA). 96% out of these 30 patients had xerophthalmia, 86.2% xerostomia, 92% positive Schirmer test, 88.24% positive Rosa Bengala test, lisamine green or Ocular Staining Score, 81.2% positive unstimulated salivary flow, 82.1% Ro(+) and 33.33% La(+). Minor salivary gland biopsy had been performed in 14 of the 30 patients, 12 with positive results. There were no statistically significant differences respect baseline characteristics when comparing the patients who developed another ARD to the ones that did not. CONCLUSIONS: Of all the patients analyzed, 4.4% presented another ARD during their follow-up. It is important to be aware of this, to make an early and proper diagnosis and treatment of our patients.

Risk Factors and Outcomes for COVID-19 in Autoimmune Inflammatory Diseases during the SARS-CoV-2 Pandemic: A Comparative Study.

BACKGROUND: Patients with autoimmune disease (AID) and coronavirus disease 2019 (COVID-19) could have higher mortality due to the co-morbidity and the use of immunosuppressive therapy. OBJECTIVES: To analyze the risk factors and outcomes of patients with AID and COVID-19 versus a control group. METHODS: A prospective cohort study included patients with and without AID and COVID-19. Patients were paired by age and sex. Clinical, biochemical, immunological treatments, and outcomes (days of hospital stay, invasive mechanical ventilation [IMV], oxygen at discharge, and death) were collected. RESULTS: We included 226 COVID-19 patients: 113 with AID (51.15 ± 14.3 years) and 113 controls (53.45 ± 13.3 years). The most frequent AIDs were Rheumatoid arthritis (26.5%), systemic lupus erythematosus (21%), and systemic sclerosis (14%). AID patients had lower lactate dehydrogenas, C-reactive protein, fibrinogen, IMV (P = 0.027), and oxygen levels at discharge (P ≤ 0.0001) and lower death rates (P ≤ 0.0001). Oxygen saturation (SaO2) ≤ 88% at hospitalization provided risk for IMV (RR [relative risk] 3.83, 95% confidence interval [95%CI] 1.1-13.6, P = 0.038). Higher creatinine and LDH levels were associated with death in the AID group. SaO2 ≤ 88% and CO-RADS ≥ 4 were risk factors for in-hospital mortality (RR 4.90, 95%CI 1.8-13.0, P = 0.001 and RR 7.60, 95%CI 1.4-39.7, P = 0.016, respectively). Anticoagulant therapy was protective (RR 0.36, 95%CI 0.1-0.9, P = 0.041). CONCLUSIONS: Patients with AID had better outcomes with COVID-19 than controls. Anticoagulation was associated with a lower death in patients with AID.