S187 – Autoimmune Indicators in Idiopathic Subglottic Stenosis

Abstract

Objectives Present new data regarding the role of autoimmune disease in Idiopathic Subglottic Stenosis. Methods Patients with ISS identified between April 2007 and February 2008 were included. Directed subglottic biopsies and serum samples were collected after IRB approval and appropriate consent. Direct immunohistochemical staining was conducted on biopsy samples and indirect immunohistochemical staining was performed on serum samples. Results 35 females were identified with ISS. The mean age was 51 (range 23–81). 12 patients underwent direct Immunofluorescence staining with biopsies taken from the stenotic region. Only 2 biopsies had adequate epithelial surface area to perform direct immunofluorescence, which was negative in both cases. Indirect immunofluorescence stains were performed on 18 samples using a monkey esophagus model and human salt split skin to identify autoimmune antibodies to epithelial and basement membrane components. For the same specimens, ELISA tests were performed for Desmoglein 1, Desmoglein 3, BP 180, and BP230 markers that are positive in various autoimmune mucocutaneous disorders. Only 2 serum specimens were weakly positive for BP180. However, 13 of 18 specimens (72%) displayed nonspecific nuclear staining indicative of a nuclear autoantibody component, suggesting a connective tissue disorder. Conclusions This work does not demonstrate clear evidence of an autoimmune mucocutaneous disorder involved in the pathogenesis of ISS; however, the non-specific antinuclear staining may indicate an association with a connective tissue disorder. Further studies are required to identify the exact role and pathogenesis that the immune system plays in ISS for future targeted therapy.