Abstract
A family including a mother with SLE and daughter with PSS is reported. The mother developed multiple purpura in the legs at age 64. When she referred to a local hospital in July 1982, she had positive antinuclear antibody, leukopenia, thrombocytopenia, proteinuria and hematuria. She was diagnosed as SLE and treated with prednisolone 60mg per day.
Thrombocytopenia was quickly controlled, and purpura disappeared by her dischange in August. In January 1986, she had polyarthralgia and positive anti-DNA antibody.
The onset of the daughters disease was at age 37 in October 1983, when she noted polyarthralgia, Raynaud's phenomenon and sausage-like swelling of fingers. At admission to our hospital in September 1984, she had proxismal scleroderma, digital pitting scars, systemic hyperpigmentation and positive ANA. Both mother and daughter had HLA DR5.
Eleven families have been reported in international literature and 2 families in Japan. In these reports, most patients showed overlapping nature, and most primary patients were PSS. Two families including our patients in Japanese contained the same DRW 11 (5).
