Autopsy case of systemic sclerosis with severe pulmonary hypertension

We report an autopsy case of a 60-year-old Japanese woman who died 27 years after the onset of systemic sclerosis and 4 years after the diagnosis of pulmonary arterial hypertension. Oral administration of bosentan was effective in improving her dyspnea but had to be stopped because of drug eruption along with fever and eosinophilia. During hospitalization for the treatment of multiple skin ulcers and gangrene, she suddenly complained of severe respiratory difficulty, followed by bradycardia, unconsciousness and cardiopulmonary arrest. The autopsy revealed concentric intimal proliferation and marked luminal obstruction in many small- and medium-sized vessels of the lungs. In addition to right ventricular hypertrophy and dilatation, similar vascular changes were also present in the myocardial tissue and the atrioventricular node. In our patient, these marked vascular changes caused pulmonary hypertension followed by the severe right heart failure. The vascular changes in the atrioventricular node were suspected as the cause of a fatal arrhythmia leading to sudden death.