MUCOUS-MEMBRANE PEMPHIGOID - A DUAL CIRCULATING ANTIBODY-RESPONSE WITH IGG AND IGA SIGNIFIES A MORE SEVERE AND PERSISTENT DISEASE

Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease f requently associated with scarring of involved clinical sites. At pres ent, therapeutic intervention in the form of immunomodulating or immun osuppressive agents is often reserved until the onset of significant i nflammation and/or early cicatrization. We have therefore studied the clinical and immunopathological findings in 67 patients with MMP in or der to try to establish a reliable prognostic indicator by which patie nts at high risk may be identified early in the disease, Inclusion cri teria were a predominantly mucosal disease and the detection of IgG an d/or C3 anti-basement membrane zone (BMZ) immunoreactants using immuno fluorescence techniques, Patients were allocated to three disease subg roups on the basis of the modality and duration of therapeutic interve ntion required to achieve effective control of disease. In addition, a t presentation and at each follow-up visit, a clinical score for sever ity of involved clinical sites was awarded and serum collected for ind irect immunofluorescence (IIF), A dual circulating anti-basement membr ane zone (anti-BMZ) antibody response with IgG and IgA was significant ly associated with a more severe and persistent disease profile (P < 0 .001). The odds ratios for requiring systemic therapy were: 11.6 among patients in whom there was a clinical score greater than or equal to 5 compared with a score < 5, and 31.3 and 66.9 among patients with IgG alone and both IgG and IgA, respectively compared with negative IIE T he findings suggest that an assessment based upon a combination of sit e severity score and the presence of circulating IgG and IgA by IIF us ing 1 mol/L salt-split human skin substrate may be considered a useful prognostic indicator.