Relationship between the in vivo localization and the immunoblotting pattern of anti-basement membrane zone antibodies in patients with bullous pemphigoid

132

100

To investigate the safety and efficacy of oral methylprednisolone combined with azathioprine sodium or mycophenolate mofetil for the treatment of bullous pemphigoid.Design: A prospective, multicenter, randomized, nonblinded clinical trial to compare 2 parallel groups of patients with bullous pemphigoid undergoing different treatments.Setting: Thirteen departments of dermatology in Germany.Patients: Patients with bullous pemphigoid (n=73) as evidenced by clinical lesions suggestive of bullous pemphigoid, signs of subepidermal blistering on histologic analysis of skin biopsy specimens, linear deposition of IgG and C3 along the dermoepidermal junction, and deposition of autoantibodies at the blister roof in split-skin analysis.Interventions: Treatment with oral methylprednisolone plus azathioprine (azathioprine group) or oral methylprednisolone plus mycophenolate mofetil (mycophenolate mofetil group). Main Outcome Measures:The cumulative total methylprednisolone doses and rates of remission. Secondary outcome measures were safety profiles and duration of remission.

Section: Discussionmentioning

confidence: 99%

A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid

Beissert¹,

Werfel²,

Frieling³

et al. 2007

132

100

“…The use of IEM allows clear differentiation [15][16][17]23,24 between EBA or VBSLE, CP, and BP, but not from herpes gestationis, which was excluded from our study. 10,14 It is very sensitive, since all patients have such immunoreactants, and fairly reproducible.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Clinical Criteria for Diagnosis of Bullous Pemphigoid

Vaillant¹

1998

171

132

To check the potential usefulness of clinical criteria for the diagnosis of bullous pemphigoid when state-of-the-art techniques such as Western immunoblotting, immunoprecipitation, and indirect immunofluorescence on salt-split skin or direct immunoelectron microscopy are not available.Design: Comparison of the clinical criteria between 2 groups (with and without bullous pemphigoid) as defined by immunoelectron microscopy used as standard criterion, in a prospective study. Multivariate logistic regression analysis was carried out by including all items that were statistically significant (at PϽ.05 level) in univariate analysis.Setting: Five dermatology departments in teaching hospitals. Patients:The 231 patients studied had subepidermal autoimmune bullous diseases with linear IgG or C3 deposits in the basement membrane zone (157 with bullous pemphigoid, 33 with cicatricial pemphigoid, 30 with epidermolysis bullosa acquisita, 5 with lupus erythem-atosus, and 6 others). A second set of patients was used to calculate predictive values. Results:The multivariate logistic stepwise analysis resulted in a final set of predictors that included only 4 items: absence of atrophic scars, absence of head and neck involvement, absence of mucosal involvement, and age greater than 70 years. No additional variables met the .05 significance level to enter into the model. If 3 of these 4 characteristics were present, a diagnosis of bullous pemphigoid could be made with a sensitivity of 90% and a specifity of 83%; these predictive values were calculated on a sample of 70 new cases.Conclusions: With an estimated incidence of bullous pemphigoid among subepidermal autoimmune bullous diseases of 80%, the presence of 3 of the 4 significant criteria allows the diagnosis of bullous pemphigoid, with a positive predictive value of 95%. Our set of' clinical criteria thus allows the diagnosis of bullous pemphigoid with good validity for both clinical practice and therapeutic trials.

“…More details can be found in Joly et al 11 Briefly, consecutive patients with incident BP were recruited if the following inclusion criteria were met: (1) clinical features suggestive of BP 6 ; (2) the typical histological pattern of BP, including a subepidermal blister on a skin biopsy specimen 24 ; and (3) linear deposits of IgG and/or C3 along the basement membrane zone as shown on results of direct immunofluorescence. 25,26 Patients were randomly assigned to receive topical or oral corticosteroids. The study was approved by the ethics committee of Seine Maritime, and written informed consent was obtained for each patient.…”

Section: Patientsmentioning

confidence: 99%

Prediction of Survival for Patients With Bullous Pemphigoid

Joly

Bénichou²,

Lok³

et al. 2005

150

131

To identify the prognostic factors of bullous pemphigoid (BP).Design: Prospective study of patients with BP included in a randomized, controlled trial.Setting: Twenty dermatology departments in France.Patients: One hundred seventy patients with BP initially treated with a 40-g/d dosage of clobetasol propionate cream (testing sample) and 171 patients initially treated with oral corticosteroids at a dosage of 0.5 or of 1.0 mg/kg per day, depending on the extent of BP (validation samples). Main Outcome Measures:The end point was overall survival during the first year after BP diagnosis. From the testing sample, associations of clinical and biological variables with overall survival were assessed using univariate and multivariate analyses. Selected predictors were included in a prognostic model. To verify that these predictors were not dependent on the treatment used, the model was then validated independently on the 2 series of BP patients treated with oral corticosteroids.Results: Median age of the BP patients included in the testing sample was 83 years. The 1-year Kaplan-Meier survival rate was 74%. From univariate analysis, the main deleterious predictors were demographic factors (ie, older age and female sex), associated medical conditions (ie, cardiac insufficiency, history of stroke, and dementia), and low Karnofsky score, which is a measure of the patient's general condition. No factors directly related to BP, in particular extent of cutaneous lesions, were shown to be related to the patients' prognosis. From multivariate analysis, only older age (P =.02) and low Karnofsky score (PϽ.001) appeared independently predictive of death. From the Cox model including these 2 predictors, the predicted 1-year survival rates were 90% (95% confidence interval [CI], 85%-96%) for patients 83 years or younger with Karnofsky score greater than 40, 79% (95% CI, 69%-90%) for patients older than 83 years with Karnofsky score greater than 40, 65% (95% CI, 50%-86%) for patients 83 years or younger with Karnofsky score of 40 or less, and 38% (95% CI, 26%-57%) for patients older than 83 years with Karnofsky score of 40 or less. Kaplan-Meier survival distributions of patients from the validation samples appeared clearly separated according to these 4 categories and were in close agreement with corresponding predicted 1-year survival rates obtained from the testing sample. Conclusions:The prognosis of patients with BP is influenced by age and Karnofsky score. These predictors are easy to use and should facilitate the management of BP.