[Lethal forms of connective tissue disease with antiribonucleoprotein antibodies (author's transl)]

Following Sharp's initial findings, the presence of antiribonucleoprotein antibodies in patients with connective tissue disease has long been considered as a sign of favourable prognosis. However, the long-term follow-up of such patients has revealed an increase in the number of severe or lethal cases. The authors report one lethal case and review 22 other published cases. Attention is drawn to the frequency of forms with onset in childhood and of scleroderma-like forms. Most deaths are due to pulmonary lesions, but the heart and kidneys may also be involved. Histological lesions of visceral vasculitis are extremely common.