Profile of autoantibody to basement membrane zone proteins in patients with mucous membrane pemphigoid: long-term follow up and influence of therapy.
Mucous membrane pemphigoid (MMP) is an autoimmune mucocutaneous blistering disease characterized by autoantibodies to components within the basement membrane zone. In this study, we report the titers of autoantibodies to antigens in the BMZ, in the sera of 13 patients, treated with intravenous immunoglobulin as monotherapy over a consecutive 18-month period. Using bovine gingiva lysate as substrate in an immunoblot assay, autoantibodies to human bullous pemphigoid antigens (BPAg1 and BPAg2), human beta4 integrin, and laminin 5 were measured. A statistically significant (P < 0.05) decline in the autoantibody titers to beta4-integrin was observed after 3.42 months of initiating the IVIg therapy. These titers were undetectable after 13 months of therapy. The titers of antibodies to BPAg1 and BPAg2 did not correlate with disease activity or response to therapy. Antibodies to laminins were not detected. In patients with MMP, autoantibody titers to beta4-integrin correlate with disease activity and response to therapy.
Department of Oral Medicine, Infection and Immunity, Harvard School of Dental Medicine, Boston, MA 02115, USA.
Shih Wei
SW
Yeh
Adnan Q
AQ
Usman
A Razzaque
AR
Ahmed
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology*; D001324:Autoantigens / Q000097:blood / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology*; D002352:Carrier Proteins / Q000097:blood / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000097:blood / Q000276:immunology; D000071821:Dystonin; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007167:Immunotherapy; D039663:Integrin beta4 / Q000097:blood / Q000276:immunology; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000097:blood / Q000276:immunology; D024041:Non-Fibrillar Collagens / Q000097:blood / Q000276:immunology; D010390:Pemphigoid, Benign Mucous Membrane / Q000276:immunology* / Q000628:therapy*; D013997:Time Factors; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D039663:Integrin beta4; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human