Taller de concordancia en la evaluación de imágenes capilaroscópicas / Reliability of nailfold videocapillaroscopy workshop

Introducción: la capilaroscopia es un método no invasivo que permite observar la microvasculatura en el área periungueal. Los resultados informados pueden ser altamente variables entre distintos observadores. A lo largo del tiempo surgieron métodos cuantitativos y semicuantitativos para mejorar la reproducibilidad. Objetivos: conocer el nivel de acuerdo intra e interobservador al informar los diferentes patrones capilaroscópicos en individuos con diferente nivel de entrenamiento. Materiales y métodos: estudio de corte transversal. Participaron médicos reumatólogos especialistas y en formación que habían realizado previamente un curso virtual de capacitación en capilaroscopia. Recibieron 40 imágenes capilaroscópicas proyectadas en una presentación de PowerPoint y debían responder a través de un cuestionario digital. Se evaluó la concordancia de respuestas intra e interobservador. Resultados: se encontró un alto nivel de concordancia global con un kappa 0,66 IC 95% (0,63-0,70) p<0,0000. También en otros grupos como reumatólogos en formación: kappa 0,65 IC 95% (0,60-0,71) p=0,0000, y médicos reumatólogos: kappa 0,67 IC 95% (0,62-0,72) p=0,0000. Conclusiones: el nivel de concordancia encontrado fue globalmente alto, independientemente del nivel de entrenamiento de los profesionales, y de ser o no reumatólogo. La concordancia fue superior cuando se comparó a quienes tenían más de 4 años de experiencia en la realización de videocapilaroscopia.

Introduction: videoapillaroscopy is a non-invasive method that allows the observation of the microvasculature in the periungual area. Reported results can be highly variable between different observers. Over time, quantitative and semi-quantitative methods emerged to improve reproducibility. Objetives: to know the level of intra and interobserver agreement when reporting the different capillaroscopic patterns in individuals with different levels of training. Materials and methods: cross section study. Specialist rheumatologists and those in training who had previously completed a virtual capillaroscopy training course participated. They received 40 capillaroscopic images projected in a PowerPoint presentation and had to issue their response through a digital questionnaire. Concordance of intra and interobserver responses was evaluated. Results: a high level of global agreement was found with a kappa 0.66 CI 95% (0.63-0.70) p<0.0000, also in other groups such as rheumatologists in training: kappa 0.65 CI 95% (0.60-0.71) p=0.0000, physicians rheumatologists: kappa 0.67 95% CI (0.62-0.72) p=0.0000. Conclusions: the level of agreement found was globally high, regardless of the level of training of the professionals, and whether or not they were a rheumatologist. Concordance was higher when compared to those who had more than 4 years of experience performing videocapillaroscopy.

Enteropatía perdedora de proteínas asociada a lupus eritematoso sistémico refractaria con buena respuesta a rituximab / Protein-losing enteropathy associated with refractory systemic lupus erythematosus with a good response to rituximab

Se presenta un caso de enteropatía perdedora de proteínas (EPP)como manifestación inicial del lupus eritematoso sistémico (LES). Se trata de una paciente de sexo femenino, de 17 años de edad, que consulta por síndrome ascítico edematoso e hipoalbuminemia. El diagnóstico de LES se estableció por la presencia de: rash malar, úlceras orales, trombocitopenia, anticuerpos antinucleares, anticardiolipinas IgM y anticoagulante lúpico positivos. Se descartó el compromiso renal y hepático como causa de hipoproteinemia. El diagnóstico de la pérdida enteral de proteínas se realizó con el Clearance de alfa 1 antitripsina. La EPP fue refractaria a distintas líneas de inmunosupresores, como corticoides, ciclofosfamida, azatioprina y ciclosporina, presentando respuesta satisfactoria y sostenida a rituximab, lo que posibilitó la reducción de corticoides y la remisión de la enfermedad por tiempo prolongado (AU)

A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission (AU)

Protein-losing enteropathy associated with refractory systemic lupus erythematosus with a good response to rituximab.

A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission.

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.