Can nailfold videocapillaroscopy images be interpreted reliably by different observers? Results of an inter-reader and intra-reader exercise among rheumatologists with different experience in this field.

Nailfold videocapillaroscopy (VCP) allows non-invasive assessment of the microcirculation. Adequate training in this field is relevant for rheumatologists. There is increasing evidence of the reliability of VCP findings among different readers. Objective: To evaluate inter- and intra-reader agreement of rheumatologists to identify normal images and systemic sclerosis (SSc) patterns on VCP ("early," "active," and "late" proposed by Cutolo et al.). Thirteen rheumatologists with different experience in nailfold VCP received training to standardize reading criteria. They rated 60 VCP images from healthy and SSc patients at baseline and 4 weeks later, using an electronic platform. The reading of an expert was considered the gold standard. Data were analyzed using Cohen's kappa for concordance and Student's t test and ANOVA to compare kappa means for inter-reader, intra-reader, and inter-pattern readings. Mean inter-reader and intra-reader kappa were 0.45 and 0.49, respectively, (moderate agreement). Kappa scores were higher among experienced vs inexperienced readers (inter-reader kappa 0.58 vs 0.34, p = 0.001, intra-reader kappa 0.65 vs 0.37, p = 0.01). Agreement was substantial (kappa = 0.61) for the identification of normal vs abnormal images and higher for the identification of active (0.48, p = 0.009) and late SSc patterns (0.56, p = 0.008) than for the early SSc pattern (0.35, p = 0.003). There is moderate agreement among rheumatologists for the identification of SSc videocapillaroscopy patterns (higher among experienced rheumatologists) and substantial agreement, regardless of previous experience in VCP, in the identification of normal and abnormal images. Agreement for the identification of active and late patterns is higher than for the early pattern.

Hemorragia alveolar difusa como forma de presentación de lupus eritematoso sistémico asociado a tiroiditis autoinmune

El lupus eritematoso sistémico es una enfermedad inflamatoria crónica de naturaleza autoinmune, de etiología desconocida en la que hay daño celular y tisular por autoanticuerpos y que cursa con un amplio espectro de manifestaciones clínicas. La hemorragia alveolar difusa es una forma de presentación poco frecuente en pacientes con lupus eritematoso sistémico, raramente debuta como una manifestación inicial de la enfermedad. Aproximadamente 2 % de todos los pacientes con lupus eritematoso sistémico presentan este cuadro, siendo su asociación con otras enfermedades de origen inmune, entre ellas la tiroiditis autoinmune un caso excepcional con elevada mortalidad

Systemic lupus erythematosus is a chronic inflammatory disease of autoimmune nature of unknown etiology in which there is cell and tissue damage that causes autoantibodies and a broad spectrum of clinical manifestations. Diffuse alveolar hemorrhage is a rare presentation in patients with Systemic lupus erythematosus, rarely it debuts as an initial manifestation of the disease. Approximately 2 % of all patients with Systemic lupus erythematosus have this disease, and its association with other immune-mediated diseases including autoimmune thyroiditis an exceptional case with high mortality

Síndrome de Evans en paciente con síndrome antifosfolipídico secundario: Desafío terapéutico

El síndrome de Evans, es caracterizado por la presencia de anemia hemolítica autoinmune y púrpura trombocitopénica, presentándose con menor frecuencia en pacientes con diagnóstico de lupus eritematoso sistémico. La asociación de estas dos entidades con el síndrome de anticuerpos antifosfolípidos se torna inusual, constituyendo un desafío diagnóstico y a la vez terapéutico para el clínico. Presentamos un paciente con lupus eritematoso sistémico, que desarrollo síndrome de Evans y síndrome antifosfolípido, complicado con hemorragia intracerebral cisternal y trombosis venosa profunda de miembros superiores

Evans syndrome is characterized by the presence of autoimmune hemolytic anemia and thrombocytopenic purpura, appearing less frequently in patients with systemic lupus erythematosus. The association of these two entities with antiphospholipid antibody syndrome becomes unusual, constituting a diagnostic challenge and therapeutic. We present a patient with systemic lupus erythematosus who developed Evans syndrome and antiphospholipid syndrome, complicated with cerebral haemorrhage and deep venous thrombosis of upper limbs