Prevalence of Non–Organ-Specific Autoantibodies in Patients with Pemphigus Vulgaris

Abstract

Pemphigus vulgaris (PV) is a potentially life-threatening, organ-specific, autoimmune, blistering disease of the skin and mucous membranes. Although several reports suggest an association between pemphigus and other autoimmune connective tissue disorders, studies that measure non-organ-specific autoantibodies are lacking. To evaluate the prevalence of antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA) antibodies, and antibodies against extractable nuclear antigens (ENAs) in PV patients. Serum samples were obtained from 59 PV patients and 50 healthy controls. Indirect immunofluorescence assays containing human epithelial cell substrates (HEp-2) and Crithidia luciliae were used to detect ANA and anti-dsDNA antibodies, respectively. A multiplexed addressable laser bead immunoassay was employed to measure autoantibodies to: Smith (Sm), ribonucleoprotein (RNP), Sjögren syndrome B (SSB/La), Sjögren syndrome A (SSA/Ro), histidyl transfer ribonucleic acid synthetase (Jo-1), topoisomerase I (Scl-70), and ribosome-P (Ribo-P) antigens. Positive ANAs were obtained in 22 of 59 (37.3%) PV patients compared with 4 of 50 (8.0%) healthy controls (p <or= .01). In both the patient and control populations, anti-dsDNA antibodies were absent. Antibodies against the ENAs were present in 1 of 59 (1.7%) PV patients compared with 0 of 50 (0%) controls. In the single ENA-positive PV patient, antibodies to SSB/La (1.7%) and SSA/Ro (1.7%) were detected. Non-organ-specific autoantibodies are prevalent in the PV population. As ANAs are detected in over one-third of PV patients, clinicians should screen for signs and symptoms of other connective tissue disease. Correlative clinical studies are warranted to determine the diagnostic, prognostic, and therapeutic significance of these findings.