Overlap syndromes in the context of shared autoimmunity

Abstract

The presence of autoimmune rheumatic diseases in several members of the same family, the concurrence of autoimmune rheumatic with non-rheumatic diseases in relatives of patients, the presence of autoantibodies in sera from healthy relatives of autoimmune-disease patients, the development of two or more autoimmune rheumatic diseases in one patient and the interplay of genetic and environmental factors leading to the presence of several autoimmune disease and/or their autoantibodies in families, is being termed “shared autoimmunity”. Herein we analyzed autoimmune rheumatic overlap syndromes in this context. We performed a retrospective analysis of the clinical, serological and radiological characteristics of patients with overlap syndromes from the Clinic of Rheumatic Diseases at the Department of Immunology and Rheumatology of the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. We found 23 patients with overlap syndromes; 13 patients with rhupus, 5 with sclerodermatomyositis, 3 with scleroderma and SLE, one with sclerodermatomyositis and SLE and one with scleroderma and MPA. Rhupus tends to develop sequentially while sclerodermatomyositis tends to appear simultaneously. The other overlap syndromes are less common and their clinical course is rather aggressive, although clinical manifestations respond to standard treatment. The second and/or third disease appears while the first one is still active, even with adequate treatment. The coexistence of autoimmune rheumatic diseases may be partially explained by the interplay of environmental factors with genes that predispose to autoimmunity in general and to manifestations of specific diseases. This is part of the concept of Shared Autoimmunity.