RESUMO
BACKGROUND: Adverse pregnancy outcomes (APO) occur in 35% of patients with pemphigoid gestationis (PG). No biological predictor of APO has been established yet. OBJECTIVES: To assess a potential relationship between the occurrence of APO and the serum value of anti-BP180 antibodies at the time of PG diagnosis. METHODS: Multicentre retrospective study conducted from January 2009 to December 2019 in 35 secondary and tertiary care centres. INCLUSION CRITERIA: (i) diagnosis of PG according to clinical, histological and immunological criteria, (ii) ELISA measurement of anti-BP180 IgG antibodies determined at the time of PG diagnosis with the same commercial kit and (iii) obstetrical data available. RESULTS: Of the 95 patients with PG included, 42 had one or more APO, which mainly corresponded to preterm birth (n = 26), intrauterine growth restriction (IUGR) (n = 18) and small weight for gestational age at birth (n = 16). From a ROC curve, we identified a threshold of 150 IU ELISA value as the most discriminating to differentiate between patients with or without IUGR, with 78% sensitivity, 55% specificity, 30% positive and 91% negative predictive value. The threshold >150 IU was confirmed using a cross-validation based on bootstrap resampling, which showed that the median threshold was 159 IU. Upon adjusting for oral corticosteroid intake and main clinical predictors of APO, an ELISA value of >150 IU was associated with the occurrence of IUGR (OR = 5.11; 95% CI: 1.48-22.30; p = 0.016) but not with any other APO. The combination of blisters and ELISA values higher than 150 IU led to a 2.4-fold higher risk of all-cause APO (OR: 10.90; 95% CI: 2.33-82.3) relative to patients with blisters but lower values of anti-BP180 antibodies (OR of 4.54; 95% CI 0.92-34.2). CONCLUSION: These findings suggest that anti-BP180 antibody ELISA value in combination with clinical markers is helpful in managing the risk of APO, in particular IUGR, in patients with PG.
Assuntos
Penfigoide Gestacional , Penfigoide Bolhoso , Nascimento Prematuro , Gravidez , Feminino , Humanos , Recém-Nascido , Penfigoide Gestacional/diagnóstico , Estudos Retrospectivos , Penfigoide Bolhoso/diagnóstico , Vesícula , Resultado da Gravidez , Colágenos não Fibrilares , Ensaio de Imunoadsorção Enzimática , Imunoglobulina G , Autoantígenos , AutoanticorposRESUMO
Pemphigus is a rare autoimmune bullous disorder involving the skin and mucosa. The disease has a chronic course. It is characterized histologically by an intraepidermal cleavage and the production of pathogenic antibodies directed against different proteins of the desmosomes, which belong to the cadherin family. The diagnosis of the type of pemphigus is made on clinical features, the level of histologic cleavage, and the identification of the antigens recognized by circulating autoantibodies using immunoblot or ELISA analysis of serum. The epidemiology and clinical, histologic, and immunologic findings of pemphigus vulgaris, pemphigus foliaceus, pemphigus vegetans, and pemphigus herpetiformis are described.