Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes / Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients

Introducción: El síndrome de pulmón encogido (SPE) es una manifestación rara del lupus eritematoso sistémico. Nuestro objetivo fue describir las características clínicas, radiológicas y funcionales de una cohorte con SPE y su evolución en el tiempo.MétodosEstudio retrospectivo entre 2009 y 2018. Se recogieron datos demográficos, clínicos, funcionales, radiológicos y de tratamiento.ResultadosDe un total de 225 pacientes, 11 presentaron SPE (prevalencia del 4,8%). Dos fueron excluidos. La edad media fue 39,33±16 años, 6 eran mujeres. Los síntomas principales fueron la disnea y el dolor pleurítico. La capacidad vital forzada media fue del 49%, la capacidad pulmonar total del 60%, la capacidad de difusión de monóxido de carbono del 66%, el factor de transferencia para el monóxido de carbono del 128%, la presión inspiratoria máxima del 66% y la presión espiratoria máxima del 82%. Todos los pacientes recibieron corticosteroides. Después de una mediana de seguimiento de 19 meses, 4 casos presentaron mejoría y 4 estabilización.ConclusionesEl SPE debe tenerse presente en todo paciente lúpico con disnea de causa no evidente. Si bien suele evolucionar con mejoría, la mayoría queda con deterioro persistente a pesar del tratamiento. (AU)

Introduction: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time.MethodsA retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected.ResultsOut of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable.ConclusionsSLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment. (AU)

[Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients]. / Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes.

INTRODUCTION: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time. METHODS: A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected. RESULTS: Out of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable. CONCLUSIONS: SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment.

Clinical Relevance of Galectin-1 and Galectin-3 in Rheumatoid Arthritis Patients: Differential Regulation and Correlation With Disease Activity.

Galectins, a family of animal lectins, play central roles in immune system regulation, shaping both innate and adaptive responses in physiological and pathological processes. These include rheumatoid arthritis (RA), a chronic multifactorial autoimmune disease characterized by inflammatory responses that affects both articular and extra-articular tissues. Galectins have been reported to play central roles in RA and its experimental animal models. In this perspective article we present new data highlighting the regulated expression of galectin-1 (Gal-1) and galectin-3 (Gal-3) in sera from RA patients under disease-modifying anti-rheumatic drugs (DMARDs) and/or corticoid treatment in the context of a more comprehensive discussion that summarizes the roles of galectins in joint inflammation. We found that Gal-1 levels markedly increase in sera from RA patients and positively correlate with erythrocyte sedimentation rate (ERS) and disease activity score 28 (DAS-28) parameters. On the other hand, Gal-3 is downregulated in RA patients, but positively correlates with health assessment questionnaire parameter (HAQ). Finally, by generating receiver-operator characteristic (ROC) curves, we found that Gal-1 and Gal-3 serum levels constitute good parameters to discriminate patients with RA from healthy individuals. Our findings uncover a differential regulation of Gal-1 and Gal-3 which might contribute to the anti-inflammatory effects elicited by DMARDs and corticoid treatment in RA patients.

Estudio multicéntrico de prevalencia de anticuerpos antirribosomal P en lupus eritematoso sistémico de comienzo juvenil comparado con lupus eritematoso sistémico del adulto / Multicentric prevalence study of anti P ribosomal autoantibodies in juvenile onset systemic lupus erythematosus compared with adult onset systemic lupus erythematosus

Objetivo. Determinar la prevalencia y correlación clínica de los anticuerpos antirribosomal P en lupus eritematoso sistémico (LES) juvenil y compararlos con LES del adulto. Métodos. Se incluyeron en el estudio 30 pacientes con LES juvenil y 92 pacientes con LES del adulto. Consideramos LES de comienzo juvenil a todos aquellos pacientes que comenzaron su enfermedad antes de los 16 años. Se consideraron las manifestaciones clínicas y serológicas que presentaron los pacientes desde el diagnóstico hasta el momento de inclusión en el estudio (manifestaciones acumuladas). El anticuerpo antirribosomal P fue evaluado mediante la técnica de enzimo-inmunoensayo (ELISA). Resultados. La presencia de antirribosomal P fue significativamente mayor en el grupo de pacientes con LES juvenil comparado con LES del adulto (26,7% vs. 6,5%; OR = 5,21 [IC95% = 1,6-16,5], p = 0,003). La alopecía (OR = 10,11; IC95% = 1,25-97) y rash cutáneo (no discoide) (OR = 4,1; IC95% = 1,25-13,89) fueron las únicas manifestaciones clínicas que se asociaron en forma estadísticamente significativa con la presencia del anticuerpo antirribosomal P. Conclusión. Este estudio confirma una mayor prevalencia de anticuerpos antirribosomal P en pacientes con LES juvenil. La alopecia y el rash cutáneo fueros las únicas manifestaciones clínicas asociadas a la presencia de antirribosomal P (AU)

Objective. To investigate the prevalence and associations with clinical manifestations of anti- P ribosomal antibodies in patients with juvenile-onset and adult-onset systemic lupus erythematosus (SLE). Methods. Clinical and serological data of 30 patients with juvenile-onset SLE (age at onset younger than 16 years old) were compared with data of 92 patients with adult-onset SLE. Symptoms occurring during the entire disease course were considered. Anti- P ribosomal antibodies were tested by ELISA. Results. Anti- P ribosomal antibodies were found significantly more often in pediatric-onset SLE patients (26.7% vs. 6.5%; OR = 5.21 [CI95% = 1.6-16.5], p = 0.003). Alopecia (OR = 10.11, CI 95% = 1.25-97) and skin rash (non discoid) (OR = 4.1, CI 95% = 1.25-13.89) were significantly associated with anti- P ribosomal antibodies. Conclusion. Anti-ribosomal P antibodies are more often found in patients with juvenile SLE. Alopecia and skin rash were the only clinical manifestations associated to anti-ribosomal P antibodies (AU)

Multicentric prevalence study of anti P ribosomal autoantibodies in juvenile onset systemic lupus erythematosus compared with adult onset systemic lupus erythematosus.

OBJECTIVE: To investigate the prevalence and associations with clinical manifestations of anti- P ribosomal antibodies in patients with juvenile-onset and adult-onset systemic lupus erythematosus (SLE). METHODS: Clinical and serological data of 30 patients with juvenile-onset SLE (age at onset younger than 16 years old) were compared with data of 92 patients with adult-onset SLE. Symptoms occurring during the entire disease course were considered. Anti- P ribosomal antibodies were tested by ELISA. RESULTS: Anti- P ribosomal antibodies were found significantly more often in pediatric-onset SLE patients (26.7% vs. 6.5%; OR=5.21 [CI95%=1.6-16.5], p=0.003). Alopecia (OR=10.11, CI 95%=1.25-97) and skin rash (non discoid) (OR=4.1, CI 95%=1.25-13.89) were significantly associated with anti- P ribosomal antibodies. CONCLUSION: Anti-ribosomal P antibodies are more often found in patients with juvenile SLE. Alopecia and skin rash were the only clinical manifestations associated to anti-ribosomal P antibodies.

Work instability in rheumatoid arthritis patients from Argentina: prevalence and associated factors.

To determine the prevalence of and associated factors to work instability (WI) in rheumatoid arthritis (RA) Argentinean patients. Observational cross-sectional study that assessing employment status in currently working RA patients. They answered the validated version of RA work instability scale (RA-WIS). High-risk WI was considered when RA-WIS was ≥17. Factors associated with high-risk WI were examined by univariable and multivariable analysis. Four-hundred and fifty RA patients were enrolled; of these, 205 patients were currently employed, but only 172 have completed questionnaires required [RA-WIS and health assessment questionnaire (HAQ-A)]. Their mean age was 49.3 ± 10.8 years; 81.3 % were female; and their mean disease duration was 8.1 ± 7.2 years. Fifty-two percent of patients were doing manual work. The mean RA-WIS score was 11.4 ± 6.8, and 41 % of patients had a high-risk WI. High-risk WI was associated with radiographic erosions (p < 0.001) and HAQ-A >0.87 (p < 0.001) in the univariable analysis, whereas in the multivariable logistic regression analysis the variables associated with a high-risk WI were as follows: HAQ-A >0.87 [odds ratio (OR) 12.31; 95 % CI 5.38-28.18] and the presence of radiographic erosions (OR 4.848; 95 % CI 2.22-10.5). In this model, having a higher monthly income (OR 0.301; 95 % CI 0.096-0.943) and a better functional class (OR 0.151; 95 % CI 0.036-0.632) were protective. Forty-one percent of RA working patients had high-risk WI. The predictors of high RA-WIS were HAQ-A ≥0.87 and radiographic erosions, whereas having a better functional class and have higher incomes were protective.

Anticuerpos antipéptidos cíclicos citrulinados como marcadores de diagnóstico y actividad en artritis reumatoidea temprana / Anti-cyclic citrullinated peptide antibodies as diagnostic and activity markers in early rheumatoid artritis

Objetivos: Determinar el valor diagnóstico de los Ac aCCP de segunday tercera generación para AR de reciente comienzo y compararloscon el valor diagnóstico del FR. Evaluar la actividad de la enfermedadmediante el score DAS28 al establecer el diagnóstico de AR. Resultados: Se analizaron los datos de 149 pacientes (75,3% mujeres y 24,7% varones). La edad media de los pacientes fue 58 ± 14. Al final del estudio, 61 (40,9%) cumplieron criterios para AR. Los valores de cribaje de estos anticuerpos demuestran una sensibilidad superior para las dos generaciones de Ac aCCP con respecto al FR, con una specificidad similar para todos los anticuerpos. La actividad de la enfermedad al momentodel diagnóstico medida por DAS28 fue similar en todos los grupos. Conclusiones: Los resultados indican que no existen diferencias estadísticamente significativas en los valores de cribaje entre los Ac anti CCP de las dos generaciones para el diagnóstico de AR. Los valores de cribaje de estos anticuerpos demuestran una sensibilidadsuperior para las dos generaciones de Ac aCCP con respecto al FR, con una especificidad similar para todos los anticuerpos. No hubo diferencias en la actividad de la enfermedad al inicio.

Objetives: To determine the diagnostic value of Ac ACCP second and third generation in the early AR compared to the diagnostic value of FR. To assess disease activity by DAS28 score to establish the diagnosis of RA.Results: We analyzed data from 149 patients (75.3% women and 24.7% males). The average age of patients was 58 ± 14. At the end of the study, 61 (40.9%) met criteria for RA. The values of screening for these antibodies demonstrated a higher sensitivity for the two generations of Ac aCCP with respect to the FR, with a similar specificity for all antibodies. Disease activity at diagnosis by DAS28 score wassimilar in all groups. Conclusions: The results indicate that there were no statistically significant differences among two generations of Ac aCCP for the diagnosis of RA, with a large difference with respect to the RF. Thevalues of screening for these antibodies demonstrated a higher sensitivity for the two generations of Ac aCCP with respect to the FR, with a similar specificity for all antibodies. The activity at the onset of the disease was similar for all the groups.

Anticuerpos antipéptidos cíclicos citrulinados como marcadores de diagnóstico y actividad en artritis reumatoidea temprana / Anti-cyclic citrullinated peptide antibodies as diagnostic and activity markers in early rheumatoid artritis

Objetivos: Determinar el valor diagnóstico de los Ac aCCP de segunday tercera generación para AR de reciente comienzo y compararloscon el valor diagnóstico del FR. Evaluar la actividad de la enfermedadmediante el score DAS28 al establecer el diagnóstico de AR. Resultados: Se analizaron los datos de 149 pacientes (75,3% mujeres y 24,7% varones). La edad media de los pacientes fue 58 ± 14. Al final del estudio, 61 (40,9%) cumplieron criterios para AR. Los valores de cribaje de estos anticuerpos demuestran una sensibilidad superior para las dos generaciones de Ac aCCP con respecto al FR, con una specificidad similar para todos los anticuerpos. La actividad de la enfermedad al momentodel diagnóstico medida por DAS28 fue similar en todos los grupos. Conclusiones: Los resultados indican que no existen diferencias estadísticamente significativas en los valores de cribaje entre los Ac anti CCP de las dos generaciones para el diagnóstico de AR. Los valores de cribaje de estos anticuerpos demuestran una sensibilidadsuperior para las dos generaciones de Ac aCCP con respecto al FR, con una especificidad similar para todos los anticuerpos. No hubo diferencias en la actividad de la enfermedad al inicio.(AU)

Objetives: To determine the diagnostic value of Ac ACCP second and third generation in the early AR compared to the diagnostic value of FR. To assess disease activity by DAS28 score to establish the diagnosis of RA.Results: We analyzed data from 149 patients (75.3% women and 24.7% males). The average age of patients was 58 ± 14. At the end of the study, 61 (40.9%) met criteria for RA. The values of screening for these antibodies demonstrated a higher sensitivity for the two generations of Ac aCCP with respect to the FR, with a similar specificity for all antibodies. Disease activity at diagnosis by DAS28 score wassimilar in all groups. Conclusions: The results indicate that there were no statistically significant differences among two generations of Ac aCCP for the diagnosis of RA, with a large difference with respect to the RF. Thevalues of screening for these antibodies demonstrated a higher sensitivity for the two generations of Ac aCCP with respect to the FR, with a similar specificity for all antibodies. The activity at the onset of the disease was similar for all the groups.(AU)