Mixed Connective Tissue Disease is an autoimmune clinical entity with characteristics of overlap syndrome including: Systemic Lupus Erythematosus, Rheumatoid Arthritis, Systemic Sclerosis, Polymyositis and Dermatomyositis. There is an active controversy as to whether it is a variant of Lupus or it is a different entity. The objective of these case reviews is to describe clinical-serological characteristics of 6 patients from AGAR clinic (Guatemalan Association against Rheumatic Diseases) that meet the criteria for this syndrome according to Alarcón-Segovia and Villareal criteria. Although the controversy persists, after an extensive review of the literature we consider that Mixed Connective Tissue Disease is not a variant of lupus, but a different entity with a different course and prognosis.
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