Follow-up study of 6 children presenting with a MCTD-like syndrome.

Since 1974 we have observed 6 children presenting with symptoms, signs and serology consistent with a MCTD-like syndrome. During this observation period (3-6 years) 3 children developed a severe disease, 2 of them contracted glomerulonephritis and one digital vasculitis. On the basis of the presenting symptoms the course of the disease was not predictable. The prognostic implications of the MCTD syndrome might therefore be questioned.