Chryssomallis F, Dimitriades A, Chaidemenos G C, Panagiotides D, Karakatsanis G
Department of Dermatology, Aristotles University, Thessaloniki, Greece.
Int J Dermatol. 1995 Jun;34(6):438-42. doi: 10.1111/j.1365-4362.1995.tb04450.x.
Patients with pemphigus vulgaris may occasionally present the highly active, widespread form of the disease, and/or maybe resistant to conventional oral steroid therapy, and/or manifest significant side effects from the prolonged use of high oral prednisone dosage. Our purpose was to evaluate the efficacy of steroid "pulse" therapy (PT) in these patients.
Eight patients were given alternate-day, one hour, infusions of 8, 9, or 10 mg/kg methylprednisolone Na succinate. Oral prednisone and a second immunosuppressive agent were simultaneously administered; these were rapidly decreased when control of the disease was achieved.
All patients initially responded well to therapy. The disease recurred in four patients after 3, 4, 9, and 16 months of remission, respectively. Three of these patients were treated again with PT and went into remission. None of the patients who received cyclophosphamide had a recurrence. One patient died of cardiac arrest, 12 days after PT. On a follow-up of 59 +/- 25 months since the last relapse, patients continue in remission.
Pulse therapy is recommended as an adjunct to the total management plan of severely affected patients with pemphigus vulgaris. The addition of cyclophosphamide may prevent the disease from recurring. Alternate-day small-quantity bolus infusions over 16-20 days, may be equally effective as the administration of 15 mg/kg/daily for 3-5 days. The risk of cardiac arrest exists even with this, less aggressive form of PT therapy. A medical history of supraventricular arrhythmias may be considered a risk factor.
寻常型天疱疮患者偶尔会出现疾病高度活跃、广泛分布的形式，和/或可能对传统口服类固醇疗法耐药，和/或因长期高剂量口服泼尼松而出现明显副作用。我们的目的是评估类固醇“冲击”疗法（PT）对这些患者的疗效。
8例患者接受隔日1小时静脉输注8、9或10mg/kg琥珀酸钠甲泼尼龙。同时给予口服泼尼松和第二种免疫抑制剂；疾病得到控制后迅速减量。
所有患者最初对治疗反应良好。4例患者分别在缓解3、4、9和16个月后疾病复发。其中3例患者再次接受PT治疗并进入缓解期。接受环磷酰胺治疗的患者均未复发。1例患者在PT治疗12天后死于心脏骤停。自上次复发以来随访59±25个月，患者持续缓解。
推荐冲击疗法作为重症寻常型天疱疮患者整体治疗计划的辅助治疗。加用环磷酰胺可预防疾病复发。在16 - 20天内隔日小剂量推注可能与每日15mg/kg连续给药3 - 5天同样有效。即使采用这种侵袭性较小的PT治疗形式，也存在心脏骤停的风险。有室上性心律失常病史可能被视为一个危险因素。
