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Research Letters |

Mortality of Bullous Pemphigoid in China FREE

Jun Li, MD; Ya-Gang Zuo, MD; He-Yi Zheng, MD
[+] Author Affiliations

Author Affiliations: Department of Dermatology and Venereology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.


JAMA Dermatol. 2013;149(1):106-108. doi:10.1001/archdermatol.2012.2994.
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Bullous pemphigoid (BP) is the most common acquired autoimmune blistering disorder that occurs mostly in elderly people. The first-year mortality has been reported to range from 6% to 41%.13 This study determined the 1-, 2-, and 5-year mortality of patients with BP in China and identified risk factors affecting survival. The mortality of patients with BP was compared with age-matched persons in the general population.

This was a retrospective cohort study. Inclusion criteria were a diagnosis of BP based on clinical characteristics and confirmed by at least a typical histologic pattern and the presence of autoantibodies against the basement membrane zone detected by direct and/or indirect immunofluorescence testing. The treatment for BP was oral corticosteroids with or without adjuvant therapy such as dapsone, cyclophosphamide, and others. Data were obtained of all patients with a discharge diagnosis of BP between January 1991 and 2011. Information on the status of patients who became unavailable for follow-up by the hospital department was obtained by telephone calls.

The mortality at 1, 2, and 5 years after first hospitalization was calculated based on the Kaplan-Meier survival estimate. Cox logistic regression in multivariate analysis was used. For the analysis, if no predefined cutoff points were available, continuous variables were categorized on the basis of the median. The ratio of the observed to expected death rates, or the standardized mortality ratio (SMR), was calculated for age categories. The study was approved by the ethics committee of Peking Union Medical College.

A total of 140 patients with BP and a follow-up time 1 year or longer were included. The median age was 67 years, and the mean (SD) age was 64.3 (13.6) years (age range, 18-93 years). The median time from onset of disease to hospitalization was 2.94 months (range, 7 days to 30 years). The median follow-up time was 3 years. The 1-year mortality was 12.9% (95% CI, 8.3%-19.6%); the 2-year mortality was 20.1% (95% CI, 14.4%-28.0%); and the 5-year mortality was 33.5% (95% CI, 25.6%-43.1%) (Figure).

Place holder to copy figure label and caption
Graphic Jump Location

Figure. Kaplan-Meier curve of overall survival of patients with bullous pemphigoid.

In the univariate analysis, several variables increased mortality (Table 1). Nonsignificant comparisons included smokers vs never smokers (P = .20), drinkers vs never drinkers (P = .33), urban vs rural population (P = .06), duration of disease longer than 2.94 months vs 2.94 months or shorter (P = .25), and erythrocyte sedimentation rate higher than 23 mm/h vs 23 mm/h or less.

Table Graphic Jump LocationTable 1. Risk Factors Predictive of Death in Patients With Bullous Pemphigoid

The SMR varied from 3.08 to 6.14 depending on age group (Table 2). Based on these data, we concluded that the mortality of patients in our BP cohort was higher than would be expected in age-matched persons in the general Chinese population.

Table Graphic Jump LocationTable 2. SMRs of Observed Deaths in Patients With Bullous Pemphigoid vs Expected Deaths in the General Chinese Population

In our study, the 1-year mortality was 12.9%, which is similar to rates reported in previously pub lished US studies4 but lower than reports in European studies. The European studies found a 1-year mortality varying between 19% and 41%. The younger age of patients with BP in our study (64.3 years) compared with the patient age in the European studies (74.0-82.6 years) might be responsible for this difference. The age distribution of the Chinese population differs from that of the European population in that Chinese persons 65 years or older represent only 6.83% of the total Chinese population, and those 80 years or older are only 0.88% of the total Chinese population.

Our SMR results are in accord with those reported in previously published European studies (SMR ranged from 2.15 to 15.3). A US study did not find a difference in mortality for patients with BP.3 Only hospitalized patients were included in our study, and time to death after first BP hospitalization was evaluated rather than time to death from BP diagnosis,3 which may explain our relatively higher SMR.

Several studies have suggested a relationship between BP and neurologic diseases.5 The presence of neurologic disease was related to elevated mortality in our study. Results from recent reports, in which neurological diseases also correlated with higher mortality, support our findings.3 The association of oral corticosteroid treatment alone with increased mortality may be because this treatment was used when patients with BP had poor general health.

Correspondence: Dr Li, Department of Dermatology and Venereology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China 100730 (lijun35@hotmail.com).

Accepted for Publication: June 27, 2012.

Author Contributions: All authors had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Li. Acquisition of data: Zuo and Zheng. Drafting of the manuscript: Li, Zuo, and Zheng. Critical revision of the manuscript for important intellectual content: Li. Statistical analysis: Li. Administrative, technical, and material support: Zuo. Study supervision: Li.

Conflict of Interest Disclosures: None reported.

Ahmed AR, Maize JC, Provost TT. Bullous pemphigoid: clinical and immunologic follow-up after successful therapy.  Arch Dermatol. 1977;113(8):1043-1046
PubMed   |  Link to Article
Roujeau JC, Lok C, Bastuji-Garin S, Mhalla S, Enginger V, Bernard P. High risk of death in elderly patients with extensive bullous pemphigoid.  Arch Dermatol. 1998;134(4):465-469
PubMed   |  Link to Article
Parker SR, Dyson S, Brisman S,  et al.  Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States.  J Am Acad Dermatol. 2008;59(4):582-588
PubMed   |  Link to Article
Colbert RL, Allen DM, Eastwood D, Fairley JA. Mortality rate of bullous pemphigoid in a US medical center.  J Invest Dermatol. 2004;122(5):1091-1095
PubMed   |  Link to Article
Li L, Chen J, Wang B, Yao Y, Zuo Y. Sera from patients with bullous pemphigoid (BP) associated with neurological diseases recognized BP antigen 1 in the skin and brain.  Br J Dermatol. 2009;160(6):1343-1345
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Graphic Jump Location

Figure. Kaplan-Meier curve of overall survival of patients with bullous pemphigoid.

Tables

Table Graphic Jump LocationTable 1. Risk Factors Predictive of Death in Patients With Bullous Pemphigoid
Table Graphic Jump LocationTable 2. SMRs of Observed Deaths in Patients With Bullous Pemphigoid vs Expected Deaths in the General Chinese Population

References

Ahmed AR, Maize JC, Provost TT. Bullous pemphigoid: clinical and immunologic follow-up after successful therapy.  Arch Dermatol. 1977;113(8):1043-1046
PubMed   |  Link to Article
Roujeau JC, Lok C, Bastuji-Garin S, Mhalla S, Enginger V, Bernard P. High risk of death in elderly patients with extensive bullous pemphigoid.  Arch Dermatol. 1998;134(4):465-469
PubMed   |  Link to Article
Parker SR, Dyson S, Brisman S,  et al.  Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States.  J Am Acad Dermatol. 2008;59(4):582-588
PubMed   |  Link to Article
Colbert RL, Allen DM, Eastwood D, Fairley JA. Mortality rate of bullous pemphigoid in a US medical center.  J Invest Dermatol. 2004;122(5):1091-1095
PubMed   |  Link to Article
Li L, Chen J, Wang B, Yao Y, Zuo Y. Sera from patients with bullous pemphigoid (BP) associated with neurological diseases recognized BP antigen 1 in the skin and brain.  Br J Dermatol. 2009;160(6):1343-1345
PubMed   |  Link to Article

Correspondence

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Mortality of Bullous Pemphigoid in China
Posted on January 30, 2013
Adria Gual MD
Dermatology Department. Hospital Clínic. Universitat de Barcelona
Conflict of Interest: None Declared
We read with interest the study by Li et al1 describing the prognosis of Bullous Pemphigoid (BP) in a large patient cohort in China. The authors report a first year mortality rate significantly lower than those reported in previous European studies. We recently conducted a similar retrospective study in our institution with a cohort of 101 BP patients and also found a first year mortality rate of 12.9%.2 However, after reading the results section, it strikes me that the authors do not provide information about how many patients had a follow-up time of at least 2 years and how many patients had a follow-up time of at least 5 years. As mentioned, the median follow-up time was 3 years. Regarding to this data and in the best scenario, the number of patients with a follow-up time of 5 years or longer would have been of 70. Consequently, 1-year mortality rate would have been calculated from a cohort of 140 patients and 5-year mortality rate would have been estimated from a cohort of 70 patients. Moreover, an indeterminate proportion of the patients lost to follow up from the first to the fifth year after diagnosis could have died. As a result, 5-year mortality would have been significantly higher. In our opinion, the 1-year and 5-year mortality rates are not comparable. Furthermore, the observed 5-year mortality rate is not very reliable. References1. Li J, Zuo YG, Zheng HY. Mortality of Bullous Pemphigoid in China. JAMA Dermatol. 2013; 149(1):106-8.2. Gual A, Mascaró JM Jr, Rojas-Farreras S et al. Mortality of Bullous Pemphigoid in the first year after diagnosis: a retrospective study in a Spanish medical centre. J Eur Acad Dermatol Venereol. 2012; Dec 22. doi: 10.1111/jdv.12065. [Epub ahead of print]
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