What is the morbidity and mortality associated with mixed connective-tissue disorder (MCTD)?

Updated: Oct 01, 2018
  • Author: Eric L Greidinger, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Long-term outcome studies have established pulmonary hypertension as the most common disease-related cause of death. [15] Immunoglobulin G (IgG) anticardiolipin antibodies are a marker for development of pulmonary hypertension. Infections are also a major cause of death.

Cardiac disease, most often pericarditis, is also common in MCTD patients, with prevalence estimates ranging from 13% to 65%. Other cardiac abnormalities include conduction abnormalities, pericardial effusion, mitral valve prolapse, diastolic dysfunction, and accelerated atherosclerosis. In three prospective studies with 13-15 years of follow-up, MCTD patients had an overall mortality rate of 10.4%, and 20% of these deaths were directly attributable to cardiac causes. [16]


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