Fatal primary pulmonary hypertension in mixed connective tissue disease

The clinical history and autopsy findings are reported of a 23 year old woman who had symptoms of mixed connective tissue disease (MCTD) for 4 years, and who died suddenly from pulmonary hypertension due to proliferative endarteritis of the lung vessels. These arterial lesions resembled those in progressive systemic sclerosis. Children and adolescents with MCTD appear to have an increased risk for getting this complication. The fatal outcome of pulmonary hypertension in MCTD as shown in this and other cases seems to warrant treatment with immunosuppressive drugs.