Objective: 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. Pa...Objective: 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-Mei er survival rate was 74%. From univariate analysis, the main deleterious predictorswere 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 (P<.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. m anagem entofBP.展开更多
Introduction. Pemphigus is a frequent chronic bullous dermatosis in Morocco that predominantly affects young women. It requires serious therapeutic management and often repeated hospitalizations. The purpose of this s...Introduction. Pemphigus is a frequent chronic bullous dermatosis in Morocco that predominantly affects young women. It requires serious therapeutic management and often repeated hospitalizations. The purpose of this study was to assess the particular impact of pemphigus on quality of life by comparing the quality of life in patients suffering from pemphigus with that of the general population using a generic instrument of measure of health related quality of life: the SF- 36, in association with a questionnaire exploring the impact of the disease on self perception, social relationship and behaviour. Participants and methods. A French version of the SF- 36 underwent 2 translations from French to Moroccan dialect, then two retrograde translations. After a pre- test, the questionnaire survey was administered by the same investigator to 30 patients who suffered from pemphigus, followed- up in the Ibn Rochd UHC in Casablanca and to 60 healthy adults. The two samples were paired according to age and sex. Results. In the pemphigus group, there was a significant decrease of mean scores of all the SF- 36 dimensions, except for physical pain and alteration in general status of health. The greatest alteration concerned the impact on physical and emotional status (P < 0.00001) and the smallest alteration concerned the perception of general health(P=0.02). The factors influencing this impact were: profession, face involvement and extent of lesions. Other results concerning the psychosocial impact of pemphigus are presented. Conclusion. Our study confirmed that pemphigus is responsible for great alterations in health related quality of life. Therefore, the management of this disease must take into account its impact on various fields of life of the patients.展开更多
文摘Objective: 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-Mei er survival rate was 74%. From univariate analysis, the main deleterious predictorswere 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 (P<.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. m anagem entofBP.
文摘Introduction. Pemphigus is a frequent chronic bullous dermatosis in Morocco that predominantly affects young women. It requires serious therapeutic management and often repeated hospitalizations. The purpose of this study was to assess the particular impact of pemphigus on quality of life by comparing the quality of life in patients suffering from pemphigus with that of the general population using a generic instrument of measure of health related quality of life: the SF- 36, in association with a questionnaire exploring the impact of the disease on self perception, social relationship and behaviour. Participants and methods. A French version of the SF- 36 underwent 2 translations from French to Moroccan dialect, then two retrograde translations. After a pre- test, the questionnaire survey was administered by the same investigator to 30 patients who suffered from pemphigus, followed- up in the Ibn Rochd UHC in Casablanca and to 60 healthy adults. The two samples were paired according to age and sex. Results. In the pemphigus group, there was a significant decrease of mean scores of all the SF- 36 dimensions, except for physical pain and alteration in general status of health. The greatest alteration concerned the impact on physical and emotional status (P < 0.00001) and the smallest alteration concerned the perception of general health(P=0.02). The factors influencing this impact were: profession, face involvement and extent of lesions. Other results concerning the psychosocial impact of pemphigus are presented. Conclusion. Our study confirmed that pemphigus is responsible for great alterations in health related quality of life. Therefore, the management of this disease must take into account its impact on various fields of life of the patients.
