Mourellou O, Chaidemenos G C, Koussidou T, Kapetis E
State Hospital for Skin and Venereal Diseases, Thessaloniki, Greece.
Br J Dermatol. 1995 Jul;133(1):83-7. doi: 10.1111/j.1365-2133.1995.tb02497.x.
We present a retrospective analysis of 48 patients with pemphigus vulgaris (PV) who were seen between 1978 and 1988. They were divided into three treatment groups: 25 (group A) received 40-100 mg of oral prednisone daily; eight (group B) received > 100 mg of prednisone daily; 15 (group C) received 40 mg of oral prednisone every other day and azathioprine 100 mg daily. A second immunosuppressive agent was subsequently added to the treatment regimen of three patients in group A and eight patients in group B. By 1989, 10% of the patients had been able to discontinue all therapy, and were in complete remission. Sixty-five per cent of patients were on maintenance therapy, but in clinical remission. Twenty-five per cent of the patients had died [eight in group A (31%) and four in group B (50%)] either as a consequence of the disease or its treatment. None of the patients in group C had died. Most of the deaths occurred during the first 2-3 months of therapy. Morbidity and mortality were related to the severity of the disease, to the maximum dose of prednisone required to induce remission, and to the presence of other diseases. Patients needing a total of 5 g or more of prednisone to induce a remission during the acute stage had a high mortality rate.
我们对1978年至1988年间诊治的48例寻常型天疱疮（PV）患者进行了回顾性分析。他们被分为三个治疗组：25例（A组）每天口服40 - 100毫克泼尼松；8例（B组）每天服用泼尼松超过100毫克；15例（C组）每隔一天口服40毫克泼尼松并每天服用100毫克硫唑嘌呤。随后，A组的3例患者和B组的8例患者在治疗方案中添加了第二种免疫抑制剂。到1989年，10%的患者能够停止所有治疗，并完全缓解。65%的患者接受维持治疗，但处于临床缓解状态。25%的患者（A组8例，占31%；B组4例，占50%）死于疾病本身或其治疗。C组无一例死亡。大多数死亡发生在治疗的前2 - 3个月。发病率和死亡率与疾病的严重程度、诱导缓解所需泼尼松的最大剂量以及其他疾病的存在有关。在急性期需要总共5克或更多泼尼松来诱导缓解的患者死亡率很高。
