Department of Odontostomatological and Maxillofacial Sciences, School of Medicine and Surgery, Federico II University of Naples, Naples, Italy.
Am J Clin Dermatol. 2010;11(2):137-45. doi: 10.2165/11530050-000000000-00000.
The pattern of clinical remission in pemphigus vulgaris patients still remains a controversial issue because of the limited data reported in the literature.
To evaluate the time to clinical remission in patients with exclusive oropharyngeal pemphigus vulgaris.
We conducted a long-term, longitudinal study in a university hospital. We treated 37 patients with oropharyngeal pemphigus vulgaris, who underwent a periodic follow-up for an average of 5.3 years, and evaluated their outcome in terms of clinical remission. The main outcome measure was the clinical outcome (assessed by objective measures of severity, extent of disease, intensity of therapy, and remission) before and after conventional immunosuppressive therapy.
Complete and long-lasting clinical remission was achieved in 35 patients (94.6%) with oropharyngeal lesions, of whom 13 (35.1%) were off therapy and 21 (56.8%) were on therapy at the last evaluation. One patient (2.7%) died following a stroke 3 years after complete remission on therapy. Partial remission was achieved in two patients (5.4%). The mean time to achieve complete clinical remission was 4.7 +/- 2.57 months after commencement of therapy. In all patients the mean disease severity score decreased from 7.81 +/- 1.35 at time of diagnosis to 1.0 +/- 0.9 at time of clinical remission (p < 0.0001 vs baseline), while the extent of the disease decreased from 2.9 +/- 1.0 to 0.27 +/- 0.45 (p < 0.0019 vs baseline) and the intensity of therapy from 4.91 +/- 0.64 to 0.70 +/- 0.57 (p < 0.0001 vs baseline). The mean duration of complete remission was 63.53 +/- 44.9 months.
In almost all patients with oropharyngeal pemphigus vulgaris it was possible to schedule a safe tapering of the conventional immunosuppressive therapy very shortly after the disease was controlled. Thus, we may conclude that: (i) the percentage of patients with oropharyngeal pemphigus vulgaris who achieved complete long-lasting clinical remission was very high; (ii) transient lesions that healed within a week were very frequent and had to be actively controlled; (iii) if treated early, most patients had a good clinical response and could achieve a disease- and drug-free clinical remission; (iv) early treatment may prevent extension or progression of disease; (v) there is a possible role for immunosuppressive agents; and (vi) a more favorable course of the disease, in terms of attainment and duration of clinical remission and a better prognosis, seemed to be related to a rapid response to therapy rather than to the initial severity and extent of the disease.
由于文献报道的有限数据，寻常型天疱疮患者的临床缓解模式仍然是一个有争议的问题。
评估仅口腔寻常型天疱疮患者的临床缓解时间。
我们在一家大学医院进行了一项长期的纵向研究。我们治疗了 37 例口腔寻常型天疱疮患者，他们平均进行了 5.3 年的定期随访，并根据临床缓解情况评估他们的结局。主要观察指标是常规免疫抑制治疗前后的临床结局（通过严重程度、疾病范围、治疗强度和缓解的客观测量来评估）。
35 例（94.6%）口腔病变患者完全且持久地达到临床缓解，其中 13 例（35.1%）停止治疗，21 例（56.8%）仍在治疗中。1 例（2.7%）患者在治疗后 3 年因中风死亡，完全缓解。2 例（5.4%）患者部分缓解。开始治疗后达到完全临床缓解的平均时间为 4.7±2.57 个月。所有患者的疾病严重程度评分从诊断时的 7.81±1.35 降至临床缓解时的 1.0±0.9（p<0.0001 与基线相比），疾病范围从 2.9±1.0 降至 0.27±0.45（p<0.0019 与基线相比），治疗强度从 4.91±0.64 降至 0.70±0.57（p<0.0001 与基线相比）。完全缓解的平均持续时间为 63.53±44.9 个月。
在几乎所有患有口腔寻常型天疱疮的患者中，在疾病得到控制后不久，即可安全地减少常规免疫抑制剂的用量。因此，我们可以得出以下结论：（i）口腔寻常型天疱疮患者达到完全持久临床缓解的比例非常高；（ii）在一周内愈合的短暂病变非常常见，必须积极控制；（iii）如果早期治疗，大多数患者有良好的临床反应，并能达到无疾病和无药物的临床缓解；（iv）早期治疗可能预防疾病的扩展或进展；（v）免疫抑制剂可能发挥作用；（vi）在达到和维持临床缓解以及预后方面，疾病的病程可能更有利，这似乎与对治疗的快速反应有关，而不是与疾病的初始严重程度和范围有关。
