Two histopathologic types of inflammatory vascular disease in MRL/Mp autoimmune mice. Model for human vasculitis in connective tissue disease.
We have recently described 2 histopathologic types of inflammatory vascular disease (IVD) in patients with Sjögren's syndrome (SS): neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). Autoimmune MRL/Mp mice, which have many features of SS, spontaneously develop IVD which is histopathologically indistinguishable from that observed in human SS patients. Both MRL/Mp-+/+ and MRL/Mp-lpr/lpr mice develop MIVD which evolves into NIVD and results in decreased survival; the transition to NIVD is accelerated by the lpr gene. The presence of the lpr gene on other genetic backgrounds does not result in a similar acceleration of IVD and associated decreased survival. Thus, the spontaneous autosomal recessive mutation lpr appears to modulate the development of IVD in a strain of mice with an underlying propensity for vasculitis. Based on our observations on IVD in SS patients and MRL/Mp mice, we propose a new model which may enhance our understanding of the immunopathogenesis of IVD in connective tissue disease.
D000818:Animals; D001327:Autoimmune Diseases / Q000150:complications / Q000473:pathology / Q000662:veterinary*; D003240:Connective Tissue Diseases / Q000150:complications / Q000473:pathology*; D004195:Disease Models, Animal; D005260:Female; D005786:Gene Expression Regulation; D006801:Humans; D007668:Kidney / Q000503:physiopathology; D008297:Male; D051379:Mice; D008815:Mice, Inbred Strains / Q000276:immunology*; D012376:Rodent Diseases / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000473:pathology; D014657:Vasculitis / Q000209:etiology / Q000473:pathology / Q000662:veterinary*