Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients

Abstract

The prognosis of bullous pemphigoid (BP), a disorder which usually affects elderly patients, is not well established and conflicting data have been reported about the mortality rate of the disease. Our objective in this study was to assess the clinical and immunological factors determining survival in a prospective series of 94 patients with BP. A cohort of 94 consecutive patients with BP (mean age +/- SD: 81 +/- 4 years) was studied over an 8-year period (1987-94) in one department and patients followed up for at least 1 year. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (i.e. linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting. Our analysis (median duration of follow-up: 5 years) showed that 37% of BP patients were dead within a year of starting treatment. The clinical or immunological factors which may influence the prognosis of BP were studied according to the criterion of death or survival by the end of the first year of treatment. None of the following factors was found to be significantly linked to the prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, or the presence of circulating basement membrane zone autoantibodies by indirect IF. An impaired general condition and a history of coronary artery disease indicated a bad prognosis. The presence of circulating autoantibodies against BP180 autoantigen but not autoantibodies against BP230, as detected by immunoblotting on epidermal extracts, was found to be significantly more frequent (60% vs. 25%) in BP patients who died within the first year of treatment (P < 0.01). We conclude that the presence of circulating autoantibodies against BP180 represents the first intrinsic prognostic factor that has been demonstrated in BP. This result supports the growing body of evidence for the pathophysiological importance of the anti-BP180 autoantibodies.

Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

“…Only one previous study provided evidence suggesting that presence of autoantibodies to BP180 by immunoblotting represented a poor prognostic factor [18]. …”

“…The first-year mortality rate varies between 10 and 44%, with significant differences between Europe and the USA [12,16,17]. Previous studies have identified a number of negative prognostic factors, such as age, low Karnofsky performance score and use of corticosteroids [16,18,19]. Immunosuppressive agents, systematic use of which has not been validated, may also affect the mortality rate [20].…”

Mortality of bullous pemphigoid: An evaluation of 223 patients and comparison with the mortality in the general population in the United States

“…5,6 The mortality of patients with BP in Europe, generally derived from larger studies, have been markedly higher, with 1-year mortality ranging from 19% to as high as 41%. [7][8][9][10][11][12][13][14] The reason for the large discrepancy in European versus US studies is unclear.…”

Positive Direct Immunofluorescence Is of Better Value than ELISA-BP180 and ELISA-BP230 Values for the Prediction of Relapse after Treatment Cessation in Bullous Pemphigoid: A Retrospective Study of 97 Patients

“…Anti-BP180 antibodies detected by immunoblotting were found to be associated with death in the first year [16]. The role of ELISA-BP180 test for predicting relapse during the first year of treatment [17] or after treatment cessation was previously described, and a threshold of 27 UA/ml was found to be associated with relapse at 12 months after treatment cessation.…”

“…The test is sensitive and specific (>90%) at the time of diagnosis [13,14] and results parallel the clinical activity of BP during treatment [15,16,17,18]. Values ≥27 arbitrary units (AU)/ml (3 times the 9 AU/ml threshold of the MESACUP BP180 and BP230 kit [MBL, Nagoya, Japan]) represent a major prognostic factor for predicting relapse after treatment cessation similar to persistent positive direct immunofluorescence (DIF) [6].…”