Abstract
Summary A subset of patients who often satisfy the ARA criteria for SLE but whose sera lack antinuclear antibodies when tested against various standard substrates is reviewed. Such a subset of patients forms a distinct clinical and serological group. The majority of these patients present with cutaneous lesions frequently seen in discoid or systemic lupus erythematosus. Involvement of other organ systems is less frequent in this group compared to ANA-positive SLE. The sera of these patients usually contain anti-Re and anti-La antibodies detectable by double immunodiffusion. While most patients deposit immunoglobulins in the lesional skin, direct immunofluorescence of uninvolved skin is positive only in approximately ten per cent of patients. The clinical course is marked by relapses and remissions and is favourable compared to ANA-positive SLE patients. Ten percent of SLE patients who are initially ANA-negative become ANA-positive on follow-up of approximately 4 years. Topical therapy alone may be helpful when only cutaneous manifestations are present. Systemic steroids and antimalarials are the mainstay of therapy in most cases.
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