Enfermedad mixta del tejido conectivosíndromes de solapamiento

Laburpena

The Mixed Connective Tissue Disease (MCTD) is a connective with common features of systemic lupus erythematosus (SLE), systemic sclerosis (ES) and polymyositis (PM) and to a lesser extent, rheumatoid arthritis (RA), along with the presence of high titers of anti-U1-ribonuleoproteínas (anti-U1-RNP). It is more common in women and in the third decade of life. There is a strong association between MCTD and HLA-DR4. Among the manifestations of the disease Raynaud's highlights, accompanied by arthralgia, myalgia, fever with positive ANA. Since it is an autoimmune disease, any organ can be affected, but the most common clinical manifestations are: edema of hands, sclerodactyly, Raynaud's phenomenon, arthralgias and polyarthritis. Regarding pulmonary manifestations, pulmonary hypertension is more severe involvement, appearing in 13% of patients, and is often the major cause of mortality in these patients. Treatment depends on the manifestations and complications of the disease. In his initial description emphasized the good clinical course and good response to glucocorticoids. Today we know that this is not entirely true, since many of these patients end up having fibrosis or pulmonary hypertension.