RESUMO
BACKGROUND: ELISA-BP180 values and direct immunofluorescence (DIF) are prognostic factors for relapse after treatment cessation in bullous pemphigoid (BP). OBJECTIVE: To determine the relevance of ELISA-BP230 antibodies for predicting relapse 6 months after treatment cessation. METHODS: We retrospectively selected patients with BP and available data from ELISA-BP180 and -BP230 and DIF performed at treatment cessation. The rate of relapse was calculated at 6 months. We compared ELISA-BP180 and -BP230 values and DIF in patients with relapse and remission. RESULTS: We included 97 patients. At 6 months, 25.6% of patients showed relapse. The proportion of patients with an ELISA-BP230 value ≥27 UA/ml was higher, but not significantly, for those with relapse than for those with remission (p = 0.11). The frequency of positive DIF findings was significantly higher for patients with relapse (p = 0.005). CONCLUSION: DIF is of better value than ELISA-BP180 and -230 tests to predict relapse after treatment cessation in BP.
Assuntos
Autoanticorpos/sangue , Autoantígenos/imunologia , Proteínas de Transporte/imunologia , Proteínas do Citoesqueleto/imunologia , Proteínas do Tecido Nervoso/imunologia , Colágenos não Fibrilares/imunologia , Penfigoide Bolhoso/sangue , Penfigoide Bolhoso/tratamento farmacológico , Administração Cutânea , Corticosteroides/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Distonina , Ensaio de Imunoadsorção Enzimática/métodos , Feminino , Técnica Direta de Fluorescência para Anticorpo , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Recidiva , Estudos Retrospectivos , Colágeno Tipo XVIIAssuntos
Doenças Autoimunes/epidemiologia , Dermatopatias Vesiculobolhosas/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/sangue , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Biomarcadores/sangue , Feminino , França/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Sistema de Registros , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/terapia , Fatores de TempoRESUMO
BACKGROUND: Rituximab (RTX) has been shown to be effective and safe for short-term treatment of severe pemphigus. Its long-term results remain unknown. OBJECTIVE: We sought to evaluate long-term RTX efficacy and safety in comparison with classic immunosuppressants for the treatment of severe pemphigus. METHODS: This retrospective study included, from 1997 to 2010, 24 consecutive patients with severe pemphigus, treated with RTX (n = 13) or systemic corticosteroids alone or combined with immunosuppressants (n = 11 control subjects). Anti-desmoglein antibodies were titered by enzyme-linked immunosorbent assay, every 3 months the first year, then at least annually. RESULTS: Among the 13 patients treated with RTX, 9 achieved complete remission 3 months after a first RTX cycle. Thereafter, 7 patients (4 with maintenance therapy) relapsed within a mean of 18 months after the last RTX cycle and received 1 or 2 additional RTX cycles. With mean follow-up at 41 months after the first RTX cycle and 28 months after the last one, all 13 patients remained in complete remission (5 patients off therapy). No severe RTX side effects occurred. Anti-desmoglein-3 autoantibodies remained positive in 7 patients, despite long-term complete remission. Long-term remission rates and immunologic profiles did not differ between patients with pemphigus according to RTX status. LIMITATIONS: This was a single-center, retrospective study. CONCLUSIONS: RTX appeared to be an effective and well-tolerated treatment for severe pemphigus at long term. However, the long-term remission rate without maintenance therapy did not differ significantly from that of control subjects. Anti-desmoglein-1 autoantibody titers were more reliable than anti-desmoglein-3 titers for long-term follow-up.