The frequency of antinuclear antibodies (ANA) and other antinuclear factors was prospectively evaluated in patients with sickle cell disease (SCD). Ten of 44 patients studied (22.7%) had positive ANA determinations at titers ≥1:40 compared to 3 of 46 healthy controls (6.5%; p < 0.03). Eight SCD patients had ANA liters of 1:160 or greater compared to none of the controls (p < 0.003). No antibodies directed against other nuclear factors were found. An analysis of the patient histories revealed no statistical differences between the ANA-positive and ANA-negative SCD patients when correlated with disease activity.

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