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Response to: Mortality Rate of Bullous Pemphigoid in a US Medical Center

2005; Elsevier BV; Volume: 124; Issue: 3 Linguagem: Inglês

10.1111/j.0022-202x.2005.23626.x

1523-1747

P. Joly, Jacques Bénichou, Philippe Saïag, Philippe Bernard, Jean‐Claude Roujeau,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

bullous pemphygoid To the Editor: Colbert et al., 2004Colbert R.L. Allen D.M. Eastwood D. Fairley J.A. Mortality rate of bullous pemphigoid in a US medical center.J Invest Dermatol. 2004; 122: 1091-1095Abstract Full Text Full Text PDF PubMed Scopus (46) Google Scholar recently reported a 1 y overall survival probability of 89% with a 95% confidence interval (CI) of 76%–94% from a retrospective series of 38 bullous pemphygoid (BP) patients managed in a single institution in the United States (US). They hypothesized that the seemingly better prognosis of BP patients in their series as compared with four European ones (Bernard et al., 1997Bernard P. Bedane C. Bonnetblanc J.M. Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: A cohort analysis of 94 elderly patients.Br J Dermatol. 1997; 136: 694-698Crossref PubMed Scopus (74) Google Scholar; Roujeau et al., 1998Roujeau J.C. 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: 465-469Crossref PubMed Scopus (149) Google Scholar; Joly et al., 2002Joly P. Roujeau J.C. Benichou J. et al.A comparison of oral and topical corticosteroids in patients with bullous pemphigoid.N Engl J Med. 2002; 346: 321-327Crossref PubMed Scopus (429) Google Scholar; Rzany et al., 2002Rzany B. Partscht K. Jung M. et al.Risk factors for lethal outcome in patients with bullous pemphigoid.Arch Dermatol. 2002; 138: 903-908Crossref PubMed Google Scholar) may be because of differences in practice patterns, because hospitalization is the standard procedure in Europe and may favor sepsis, whereas most patients in their series were outpatients. We question their hypothesis on the following grounds. First, we note that the 1 y survival probability in Colbert et al was obtained from only four deaths. It seems problematic to compare figures from such a small single center retrospective study in which patients received widely variable treatment regimens (as underlined in the Editorial bySwerlick and Korman, 2004Swerlick R.A. Korman N.J. Bullous pemphigoid: What is the prognosis? (Editorial).J Invest Dermatol. 2004; 122: 1091-1092Abstract Full Text Full Text PDF PubMed Scopus (78) Google Scholar), with those from four large European studies that included more than 600 patients overall, two of them being prospective studies with treatment defined in an homogeneous and standardized manner. Second, it seems surprising that only 38 BP patients were managed at the Medical College of Wisconsin Affiliated Hospitals within a period of 5 y and 4 mo. Indeed, the web site of the Medical College of Wisconsin (http://www.mcv.edu/) evaluates the population of the greater Milwaukee area at 1.4 million. Based on the estimated incidence of BP of 10 cases per million population in the US reported by Colbert et al, 75 incident BP cases would have been expected from the referral area during the time of their study. Thus, the recruitment in their series corresponded to about half of what was expected. One possible explanation for this discrepancy is that the more disabled and older patients, who are at higher risk of dying in our experience, were managed outside the Medical College of Wisconsin Affiliated Hospital. Whatever the precise explanation for this discrepancy, it points to a possible selection of the healthier patients in the series of Colbert et al. Thus, it would seem crucial to understand who were the missing patients, where and how they were treated, and what their outcome was. Third, the mean age of patients from the series of Colbert et al was 77 y, as compared with 81 y in our recent clinical trial (Joly et al., 2002Joly P. Roujeau J.C. Benichou J. et al.A comparison of oral and topical corticosteroids in patients with bullous pemphigoid.N Engl J Med. 2002; 346: 321-327Crossref PubMed Scopus (429) Google Scholar). Because the probability of death of the French population with the same age and sex distribution as our patients has been estimated in the range 7%–10% per year (Roujeau et al., 1998Roujeau J.C. 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: 465-469Crossref PubMed Scopus (149) Google Scholar), this age difference may account for the differences observed in survival between the series of Colbert et al and ours. Fourth, because of the retrospective design of their study, Colbert et al were not able to assess patients' general condition accurately. In our recent prospective study including more than 300 patients treated homogeneously with a standardized treatment regimen, we found that the Karnofsky score (which is a measure of a patient's general condition on a scale ranging from 0 to 100) was the main prognostic factor of BP, in addition to age (Joly et al., 2005Joly P. Roujeau J.C. Benichou J. et al.Prediction of survival for patients with bullous pemphigoid.Arch Dermatol. 2005Crossref Scopus (125) Google Scholar). A significant proportion of BP patients included in the four recent European studies were in poor general condition. For instance, the mean Karnofsky score of BP patients included in our recent clinical trial was 64; one-third of our patients were bedridden and had a Karnofsky score of 40 or less (Joly et al., 2002Joly P. Roujeau J.C. Benichou J. et al.A comparison of oral and topical corticosteroids in patients with bullous pemphigoid.N Engl J Med. 2002; 346: 321-327Crossref PubMed Scopus (429) Google Scholar). It would be key to account for initial Karnofsky scores of patients in the study by Colbert et al upon comparing their survival with that from patients in European studies. Finally, to test the hypothesis of Colbert et al that apparent differences in survival may be because of lower hospitalization rates in their series, we compared the overall survival of the 39 BP patients initially managed as outpatients with that of the 302 patients who were initially hospitalized in our recent clinical trial (Joly et al., 2002Joly P. Roujeau J.C. Benichou J. et al.A comparison of oral and topical corticosteroids in patients with bullous pemphigoid.N Engl J Med. 2002; 346: 321-327Crossref PubMed Scopus (429) Google Scholar). We observed very similar 1 y overall survival rates of 72% (95% CI 58%–86%) and 67% (95% CI 62%–73%), respectively. This lack of significant difference in survival between the two groups (p=0.65, log-rank test) suggests that differences in the initial management of BP patients were not responsible for any major differences in survival. In conclusion, we think that the older age of European BP patients and their poorer general condition are more likely responsible for differences in survival between the US and Europe, rather than differences in practice patterns. Differences in hospital care systems may explain differences in referral patterns between the US and Europe. Large prospective multicenter studies from the US would be useful to improve our prognostic knowledge of BP and allow meaningful comparisons between the US and Europe. The authors thank Richard Medeiros, Rouen University Hospital Medical Editor, for his valuable advice in editing this manuscript.