Familial progressive systemic sclerosis (scleroderma): immunological analysis of two patients and six siblings from a single kindred.

Immunological analysis was carried out in eight siblings two of whom have progressive systemic sclerosis (PSS). No uniform disturbance in any immunological function common to the two patients or to all siblings could be found. The two PSS patients, one healthy sister with some immunological abnormalities and a healthy brother have the same HLA genotype: A2,B8/A3,B14(15). The findings indicate that the genetical background or immunological disturbancies are not the only predisposing factors for the familial progressive systemic sclerosis.