In the course of a prospective study of 165 patients with SLE a subgroup of eight patients with active SLE yet with persistently negative tests for ANF and LE cells was identified. These patients were characterized by a photosensitive skin rash with a negative lupus band test, a high incidence of arthritis, mild form of renal disease, and a positive family history for connective tissue disease. Antibodies to DNA and cytoplasmic antigens were detected in a few.