RESUMEN
The activation threshold of B cells is tightly regulated by an array of inhibitory and activator receptors in such a way that disturbances in their expression can lead to the appearance of autoimmunity. The aim of this study was to evaluate the expression of activating and inhibitory molecules involved in the modulation of B cell functions in transitional, naive, and memory B-cell subpopulations from systemic sclerosis patients. To achieve this, blood samples were drawn from 31 systemic sclerosis patients and 53 healthy individuals. Surface expression of CD86, MHC II, CD19, CD21, CD40, CD22, Siglec 10, CD35, and FcγRIIB was determined by flow cytometry. IL-10 production was evaluated by intracellular flow cytometry from isolated B cells. Soluble IL-6 and IL-10 levels were measured by ELISA from supernatants of stimulated B cells. Systemic sclerosis patients exhibit an increased frequency of transitional and naive B cells related to memory B cells compared with healthy controls. Transitional and naive B cells from patients express higher levels of CD86 and FcγRIIB than healthy donors. Also, B cells from patients show high expression of CD19 and CD40, whereas memory cells from systemic sclerosis patients show reduced expression of CD35. CD19 and CD35 expression levels associate with different autoantibody profiles. IL-10(+) B cells and secreted levels of IL-10 were markedly reduced in patients. In conclusion, systemic sclerosis patients show alterations in the expression of molecules involved in B-cell regulation. These abnormalities may be determinant in the B-cell hyperactivation observed in systemic sclerosis.
RESUMEN
Intestinal pneumatosis is a rare complication that can occur in systemic sclerosis (ES), its pathogenesis is not entirely specified and is characterized by the presence of gas in the submucosa wall and / or bowel subserosa. For a 37 year old woman presented with a diagnosis of diffuse variety EN who consults repeatedly by pain, bloating and intermittent episodes of chronic diarrhea associated with weight loss. The imaging study revealed an intestinal pneumatosis and pneumoperitoneum as the source of the picture...
La neumatosis intestinal es una complicación rara que puede presentarse en la Esclerosis Sistémica (ES), su etiopatogenia no está del todo precisada y se caracteriza por presencia de gas en la pared submucosa y/o subserosa del intestino. Se presenta el caso de una mujer de 37 años, con diagnóstico de ES variedad difusa quien consulta en repetidas ocasiones por dolor, distensión abdominal y episodios de diarrea crónica intermitente asociado a disminución de peso. El estudio con imágenes reveló una neumatosis intestinal y neumoperitoneo como origen del cuadro...
