Nephrogenic systemic fibrosis in a patient with a p-ANCA systemic vasculitis

La fibrosis sistémica nefrogénica (FSN) es una entidad de origen desconocido caracterizada por un incremento de la fibrosis cutánea. La mayoría de los casos se han descrito en pacientes con fracaso renal agudo o crónico. Las alteraciones cutáneas incluyen pápulas y placas dérmicas engrosadas e induradas distribuidas por las extremidades y el tronco. El estudio histológico pone de manifiesto un incremento en la dermis del número de células tipo fibroblasto acompañado de depósito de mucina. En la mayoría de los casos existe el antecedente reciente de exposición a agentes de contraste tipo gadolinio. Describimos una paciente que presenta los hallazgos clínicos y patológicos característicos de la FSN después de un fracaso renal agudo en el contexto de una vasculitis asociada a anticuerpos anticitoplasma de los neutrófilos (AU)

Nephrogenic systemic fibrosis (NSF) is a fibrosing skin condition of unknown origin. Most cases have been described in patients with acute or chronic renal failure. The cutaneous changes include firm and thickened, indurate skin plaques and papules on the extremities and trunk. Histopathology typically shows an increase in dermal fibroblast-like cells associated with mucin deposition. Previous exposition to gadolinium-based contrast agents was closely associated with its onset. We described a patient with the clinical and pathologic picture of NSF presented after an acute renal failure in the course of a perinuclear antineutrophil cytoplasmic antibodies associated systemic vasculitis (AU)

Nephrogenic systemic fibrosis in a patient with a p-ANCA systemic vasculitis.

Nephrogenic systemic fibrosis (NSF) is a fibrosing skin condition of unknown origin. Most cases have been described in patients with acute or chronic renal failure. The cutaneous changes include firm and thickened, indurate skin plaques and papules on the extremities and trunk. Histopathology typically shows an increase in dermal fibroblast-like cells associated with mucin deposition. Previous exposition to gadolinium-based contrast agents was closely associated with its onset. We described a patient with the clinical and pathologic picture of NSF presented after an acute renal failure in the course of a perinuclear antineutrophil cytoplasmic antibodies associated systemic vasculitis.

MICA response to gliadin in intestinal mucosa from celiac patients.

MHC class I chain-related gene A (MICA), a putative independent susceptibility gene in autoimmune diseases, encodes a surface protein present in epithelial cells that binds to NKG2D, an activating receptor of NK, alphabeta and gammadelta T cells, and could function as a stress-inducible activator of the innate immune response. There is no evidence of a long-term implication of MICA in the celiac autoimmune process. However, it could be that gliadin activation of MICA occurs only during the initial stages of the disease. In order to determine whether MICA is activated in response to gliadin in patients with celiac disease (CD), small intestinal mucosa biopsy samples from ten long-standing celiac patients on a gluten-free diet and from five non-celiac individuals were incubated with and without gliadin for 4 h. Total RNA was purified and MICA, IFNG and NKG2D mRNA were quantified by fluorescent real-time RT-PCR. Expression levels were calculated relative to GAPDH. MICA expression was detected in both patients and controls, but incubation with gliadin induced a strong increase in samples from the treated CD group compared with the non-CD controls (P=0.028), while no differences were observed for IFNG or NKG2D mRNA levels. The gliadin-provoked over-expression of MICA in "normalized" tissues from CD patients suggests a role for this stress-induced activator of the immune response in the early stages of organ-specific autoimmune destruction, probably preceding the onset of inflammation.