Chapter 17 - Mixed Connective Tissue Disease

Mixed connective tissue disease (MCTD) was first described in 1972 by Sharp et al. as a disease with overlapping features of systemic lupus erythematosus, systemic sclerosis, and polymyositis associated with high titers of hemagglutinating antibody to the ribonuclease-sensitive component (ribonucleoprotein) of extractable nuclear antigen. Recognition of gastrointestinal manifestation in patients with MCTD is important because they may have special diagnostic, therapeutic, and prognostic implications that require consideration of appropriate management. For a long time, this entity had not gained widespread recognition despite having distinct clinical, serological, and immunoregulatory profiles. This may explain the scarcity of studies focused on clinical manifestations, particularly gastrointestinal in MCTD patients.