Two histopathologic types of inflammatory vascular disease in MRL/Mp autoimmune mice. Model for human vasculitis in connective tissue disease

Arthritis Rheum. 1985 Oct;28(10):1146-55. doi: 10.1002/art.1780281011.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / pathology
  • Autoimmune Diseases / veterinary*
  • Connective Tissue Diseases / complications
  • Connective Tissue Diseases / pathology*
  • Disease Models, Animal*
  • Female
  • Gene Expression Regulation
  • Humans
  • Kidney / physiopathology
  • Male
  • Mice
  • Mice, Inbred Strains / immunology*
  • Rodent Diseases / pathology*
  • Sjogren's Syndrome / pathology
  • Vasculitis / etiology
  • Vasculitis / pathology
  • Vasculitis / veterinary*