Spontaneous murine lupus-like syndromes. Clinical and immunopathological manifestations in several strains.

TLDR

MRL/1 and BXSB male mice develop a systemic lupus erythematosus–like disease that is more acute than that seen in NZB × W mice. The study aims to exploit multiple inbred murine strains with SLE‑like syndromes to enable experimental manipulations that clarify the disease’s nature and causes. All three strains display lymphoid hyperplasia, B‑cell hyperactivity, autoantibodies, circulating immune complexes, complement consumption, immune‑complex glomerulonephritis with gp70 deposition, and thymic atrophy, demonstrating that murine SLE is a single disease comparable to human SLE, with variations arising from distinct genetic or pathophysiologic factors that converge on common immunopathologic mechanisms.

Abstract

MRL/1 and BXSB male mice have a systemic lupus erythematosus (SLE)-like disease similar to but more acute than that occurring in NZB X W mice. The common elements of lymphoid hyperplasia, B-cell hyperactivity, autoantibodies, circulating immune complex (IC), complement consumption, IC glomerulonephritis with gp70 deposition, and thymic atrophy were found in all three kinds of SLE mice. On the basis of these common elements, SLE seen in these mice can be considered a single disease in the same sense that human SLE is one disease. The differences in the SLE expressed in the different mice are no greater than those found in an unselected series of humans with SLE. However, the significant quantitative and qualitative variations in abnormal immunologic expression suggest that different constellations of factors, genetic and/or pathophysiologic, may operate in the three murine strains and that each constellation is capable of leading, via its particular abnormal immunologic consequences, to the activation of common immunopathologic effector mechanisms that cause quite similar SLE-like syndromes. From an experimental point of view, the availability of several inbred murine strains of commonplace histocompatibility types that express an SLE-like syndrome makes possible innumerable manipulations which should help to elucidate the nature and cause(s) of this disorder.