Validación del ClinESSDAI para Argentina / Validation of ClinESSDAI for Argentina

Introducción: recientemente, en Europa y en idioma inglés, se ha desarrollado el Clinical EULAR Sjögren's Syndrome Disease Activity Index (ClinESSDAI) para evaluar la actividad en pacientes con síndrome de Sjögren primario (SSp). Objetivos: validar el ClinESSDAI en pacientes con SSp en Argentina. Materiales y métodos: estudio de corte transversal. Se utilizó la versión en castellano del EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) validada en Argentina. Para evaluar la validez del constructo, se usó la escala visual análoga (EVA) desarrollada por un reumatólogo experto por dominio del ClinESSDAI y de la EVA global para el puntaje total del ClinESSDAI, mientras que otro profesional en la materia realizó el ESSDAI y ClinESSDAI. Para analizar la reproducibilidad, se estudió a un subgrupo de pacientes, sin mediar cambios en el tratamiento ni en la condición clínica, 10 días después de la evaluación basal. Todos los médicos examinaron a los pacientes desconociendo la evaluación de los demás colegas. Resultados: se incluyeron 47 pacientes con SSp. La correlación entre la EVA global y el ClinESSDAI fue muy buena (Rho 0,7), así como la correlación de la EVA y el ClinESSDAI de cada dominio. El coeficiente de correlación intraclase (CCI) entre el ESSDAI y el ClinESSDAI fue de 0,98. La reproducibilidad fue de 0,93. Conclusiones: el ClinESSDAI es una herramienta válida y reproducible en nuestra población, equiparable al ESSDAI.

Introduction: the Clinical EULAR Sjögren's Syndrome Disease Activity Index (ClinESSDAI) has recently been developed in Europe and in the English language to evaluate activity in patients with primary Sjögren's syndrome (pSS). Objectives: validate the ClinESSDAI in patients pSS in Argentina. Materials and methods: a cross-sectional study. The Spanish version of the ESSDAI, validated in Argentina, was used. To evaluate construct validity, the Visual Analog Scale (VAS) was used, performed by an expert rheumatologist per ClinESSDAI domain, and the global VAS was used for the total score of the ClinESSDAI, while another professional performed the ESSDAI and ClinESSDAI. To evaluate reproducibility, a subgroup of patients was evaluated without changes in treatment or clinical condition 10 days after the baseline evaluation. All physicians were blind to each other's evaluation. Results: 47 patients with pSS were included. The correlation between global VAS and ClinESSDAI was very good (Rho 0.7), as well as the correlation of the VAS and ClinESSDAI of each domain. The intraclass correlation coefficient (ICC) between ESSDAI and ClinESSDAI was 0.98. The reproducibility was 0.93. Conclusions: the ClinESSDAI is a valid and reproducible tool in our population, comparable to the ESSDAI.

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

Abstract 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. Key points Patients with primary Sjögren's Syndrome may develop another connective tissue disease during follow-up. The most frequently connective tissue disease developed during follow-up in the population of patients with primary Sjogren's Syndrome studied was rheumatoid arthritis. It is important to be aware of this to make an early and proper diagnosis.

Expresión clínica del Síndrome de Sjögren primario en pacientes adultos diagnosticados a edad menor o igual a 35 años versus mayores de 35 años / Clinical expression of primary Sjögren's syndrome in adult patients diagnosed at an age less than or equal to 35 years versus those older than 35 years

Objetivo: Describir y comparar las manifestaciones clínicas en pacientes adultos diagnosticados con Síndrome de Sjögren primario (SSp) a edad menor o igual a 35 años versus mayores a 35 años. Materiales y métodos: Se incluyeron pacientes mayores de 18 años de edad, con diagnóstico de SSp de acuerdo a los criterios de clasificación ACR - EULAR 2002/2016, registrados en la base de datos GESSAR (Grupo de Estudio Síndrome de Sjögren Sociedad Argentina de Reumatología). Resultados: Se incluyeron 665 pacientes. Cien (15,04%) con edad al diagnóstico ≤ 35 años, 92% mujeres. El promedio de edad del grupo > 35 años, fue de 54 + 11 años, 96% mujeres. Se encontraron diferencias estadísticamente significativas entre < 35 años vs > 35 años, en xeroftalmia (90,72% vs 95,64%, p: 0,04) y xerodermia (42,35% vs 57,36%, p: 0,03) y en los siguientes dominios del ESSDAI (EULAR Activity Index for primary Sjögren's syndrome): sistema nervioso periférico (4,05 vs 11,32, p: 0,03), respiratorio (6% vs 15,40%, p: 0,01) y renal (6% vs 1,59%, p: 0,02). Conclusión: Nuestro estudio sugiere un menor compromiso glandular en pacientes con SSp diagnosticados a menor edad, sin un patrón diferencial característico en cuanto al compromiso sistémico.

Objective: To describe and compare the clinical manifestations, in adult patients diagnosed with primary Sjögren's Syndrome at age less than or equal to 35 years versus those over 35 years of age. Materials and Methods: We analyzed the data of patients older than 18 years, with diagnosis of primary Sjögren's syndrome (American - European criteria 2002), included in the GESSAR database (Sjögren Syndrome Study Group of the Argentine Society of Rheumatology). Results: 665 patients were included. One hundred of them with an age at diagnosis less than or equal to 35 years and with a mean age at diagnosis of 29 + 4 years, 92% of them women. The average age at diagnosis of the group over 35 years was 54 + 11 years, 96% women. Statistically significant differences were found between less than or equal to 35 years vs over 35 years, in xerophthalmia (90.72% vs 95.64%, p: 0.04) and xeroderma (42.35% vs 57.36% , p: 0.03), and in the following domains of ESSDAI (EULAR Activity Index for primary Sjögren's syndrome): peripheral nervous system (4.05 vs 11.32, p: 0.03), respiratory (6% vs 15.40%, p: 0.01) and renal (6% vs 1.59%, p: 0.02). Conclusion: Our study suggests less glandular involvement in patients with pSS diagnosed at a younger age, without a characteristic differential pattern regarding systemic involvement.

Asociación entre Artritis Reumatoidea y otras enfermedades autoinmunes / Association between Rheumatoid Arthritis and other autoimmune diseases

Objetivos: Determinar la frecuencia de enfermedades autoinmunes (EAI) en pacientes con Artritis Reumatoidea (AR) y comparar la frecuencia de EAI entre pacientes con AR y sin AR ni otra EAI reumatológica. Material y Métodos: Estudio multicéntrico, observacional, analítico, retrospectivo. Se incluyeron pacientes consecutivos con AR (ACR/EULAR 2010) y como grupo control pacientes con diagnóstico inicial de Osteoartritis primaria (OA). Resultados: Se incluyeron 1549 pacientes: 831 con AR (84% mujeres, edad media 55.2 años [DE 13.6]) y 718 con OA (82% mujeres, edad media 67 años [DE 11.1]). La frecuencia de EAI en el grupo AR fue del 22% (n=183). Estos presentaron mayor frecuencia de EAI reumatológicas (9.4 vs 3.3%, p< 0.001), y menor frecuencia de EAI no reumatológicas que aquellos con OA (15.3 vs 20.5, p=0.007). La EAI reumatológica más prevalente fue el Síndrome de Sjögren, el cual fue más frecuente en el grupo AR (87.2 vs 29.2%, p< 0,001). La frecuencia de EAI reumatológicas en los pacientes con AR fue mayor en la forma erosiva (11 vs 6.8%, p=0.048). Conclusión: La frecuencia de EAI en los pacientes con AR fue del 22%, en quienes predominaron las de etiología reumatológica mientras que, las no reumatológicas predominaron en pacientes con OA.

Objectives: To determine the frequency of autoimmune diseases (AID) in Rheumatoid Arthritis (RA) patients and to compare this frequency between patients with and without RA or other rheumatologic AID. Methods: Multicenter, observational, analytical, retrospective study. Consecutive patients with diagnosis of RA (ACR/EULAR 2010) were included. Patients with initial diagnosis of primary ostearthritis (OA) were used as control group. Results: A total of 1549 patients were included: 831 RA (84% women, mean age 55.2 [±13.6]) and 718 OA (82% women, mean age 67 [± 11.1]). The frequency of AID in the RA group was 22% (n=183). RA patients showed higher frequency of rheumatologic AID (9.4 vs 3.3%, p< 0.001), and lower frequency of non-rheumatologic AID than OA patients (15.3 vs 20.5%, p= 0.007). The most prevalent rheumatic AID was Sjögren's Syndrome, which was more frequent in the AR group (87.2 vs 29.2%, p<0.001). The frequency of rheumatologic AID in RA patients was higher in those with erosive RA (11 vs 6.8%, p=0.048). Conclusion: The frequency of AID in RA patients was 22%. Rheumatologic AID were more frequent in RA patients, whereas non-rheumatologic AID prevailed in OA patients.

Manifestaciones cutáneas extraglandulares en pacientes con síndrome de Sjögren primario / Extraglandular cutaneous manifestations in patients with primary Sjögren's syndrome

RESUMEN Objetivos: Describir la frecuencia de manifestaciones cutáneas extraglandulares en pacientes con síndrome de Sjögren primario. Determinar el perfil clínico y de laboratorio de los pacientes que presentan estas manifestaciones en comparación con aquellos que no las presentan. Materiales y métodos: Se analizaron los datos de los pacientes incluidos en la base GESSAR (Grupo de Estudio Síndrome de Sjögren de la Sociedad Argentina de Reumatología). Para la comparación entre grupos, los controles se seleccionaron en forma aleatoria con una relación casos: controles de 1:4. A su vez, se compararon los pacientes con púrpura con los controles. Resultados: Sesenta y siete (14,1%) de los 474 pacientes incluidos en la base de datos tuvieron manifestaciones cutáneas extraglandulares. De ellos, el 58% tuvo púrpura. La artritis, la neuropatía, el descenso de C3 y de C4, y la crioglobulinemia fueron estadísticamente más frecuentes en los casos en comparación con los controles; sin embargo, no se encontró asociación independiente con ninguna de estas variables. En lo que respecta a púrpura, la artritis, la neuropatía periférica, la anemia, el descenso de C3 y de C4, anti-La y crioglobulinemia fueron estadísticamente más frecuentes en comparación con los controles. Solo el descenso de C4 y la positividad de crioglobulinas se asociaron en forma independiente a la presencia de púrpura. Conclusión: El 14% de los pacientes presentaron manifestaciones cutáneas extraglandulares. La púrpura fue la manifestación más frecuente. Esta se asoció en forma independiente con el descenso de C4 y la presencia de crioglobulinas.

ABSTRACT Objectives: To describe the frequency of extra-glandular cutaneous manifestations in patients with primary Sjögren's syndrome. To determine the clinical and laboratory profile of patients who present with these manifestations compared to those who do not. Materials and methods: A study was made of patients included in GESSAR database (Sjögren Syndrome Society of Argentina Rheumatology Study Group) were analyzed. For the comparison between groups, the controls were randomly selected, with a case:control ratio of 1:4. Patients with purpura were compared with controls. Results: A total of 67 (14.1%) of the 474 patients included in the database had extra-glandular cutaneous manifestations. Of them, 58% had purpura. Arthritis, neuropathy, a decrease in C3 and C4 levels, and the presence of cryoglobulins, were statistically more frequent in cases compared to controls, although there was no independent association found with any of these variables. As regards purpura, arthritis, peripheral neuropathy, anaemia, decrease in C3 and C4, anti-La, and cryoglobulinemia were statistically more frequent compared to controls. Only the decrease in C4, and the presence of cryoglobulins were independently associated with the presence of purpura. Conclusion: Extra-glandular cutaneous manifestations were observed in 14% of the patients. Purpura was the most frequent cutaneous manifestation. This was independently associated with decreased C4 and the presence of cryoglobulins.