[MCTD--mixed connective tissue disease].
Mixed connective tissue disease is a disease entity characterized by overlapping symptoms of lupus erythematosus (LE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA). Diagnostic criteria include high titers of antibodies against U1RNP as well as the presence of at least 3 of 5 of the following clinical features: edema of hands, synovitis, myositis, Raynaud phenomenon and acroscierosis. In terms of the pathogenesis, genetic as well as infectious (viral) factors appear to play a role. The acceptance of MCTD as a distinct disease entity is controversial. Terms such as "undifferentiated connective tissue disease" or "overlapping syndromes" are not helpful. One-quarter of MCTD patients transform into LE, while one-third progress to SSc. Therapeutic recommendations are glucocorticoids in combination with immunosuppressive agents and endothelin receptor antagonists. Double blind studies are not available. The prognosis is relatively good. Causes of death include pulmonary hypertension, infections and both pulmonary and cardiac failure.
Haustein
Uwe-Frithjof
UF
Klinik für Dermatologie, Venerologie und Allergologie der Universität Leipzig. ufh@medizin.uni-leipzig.de
D003937:Diagnosis, Differential; D004338:Drug Combinations; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008947:Mixed Connective Tissue Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology; D017410:Practice Guidelines as Topic; D010818:Practice Patterns, Physicians'; D016896:Treatment Outcome
D004338:Drug Combinations; D005938:Glucocorticoids; D007166:Immunosuppressive Agents