Triad of glomerulonephritis, antinuclear antibodies, and positive skin immunofluorescence. Variant of systemic lupus erythematosus

We are reporting findings in 13 patients who presented with glomerulonephritis without evidence of systemic disease, but who were found to have positive antinuclear antibody results and immunoglobulin and/or complement deposits at the dermal-epidermal junction of normal skin not exposed to light. There was no evidence of other organ involvement, and serologic tests for systemic lupus erythematosus (SLE) gave negative results. The renal disease is characterized by severe proteinuria, focal or diffuse proliferative glomerular lesions on biopsy, with variable patterns of immunoglobulin deposits. No clinical manifestations or serologic results typical of SLE have developed during prolonged observation. HLA phenotyping carried out in eight of the 13 patients revealed DR2 or DR3 alloantigens or both in seven of the eight patients, an incidence similar to that in patients with overt SLE. Because of the specificity of the skin biopsy immunofluorescence, the similarity of HLA-DR antigens, and a favorable response of the renal disease to therapy, we believe that these patients have a variant of SLE.