Apalla Zoi, Chloros Diamantis, Argyropoulou Paraskevi, Georgopoulos Dimitrios, Patakas Dimitrios, Spanos Dimitrios, Chaidemenos George
Department of Dermatology, State Hospital of Thessaloniki, Greece.
J Dermatol Case Rep. 2011 Mar 26;5(1):1-4. doi: 10.3315/jdcr.2011.1061.
Pemphigus vulgaris belongs to a group of rare, autoimmune, blistering disorders, requiring intensive immunosuppressive therapy. Lung infections are common in patients with pemphigus vulgaris.
To evaluate pulmonary function in patients with pemphigus vulgaris, in order to detect possible factors that might predispose PV patients to lung damage.
Ten patients first diagnosed with severe pemphigus vulgaris and 10 healthy individuals,were included in the study. Pulmonary function testing, blood gas analysis and quantification of α1-antitrypsin serum levels were performed.
Mild reduction of CO diffusing capacity was recorded, compared to the mean predicted normal value. DLCOSB (single-breath carbon monoxide diffusing capacity test) values did not significantly differ between patients with pemphigus vulgaris and healthy controls, while differences regarding DLCO/VA (VA: Alveolar Volume) were statistically significant. Alpha 1-antitrypsin serum levels were decreased (<2.0g/L) in 60% (6/10) of patients with pemphigus vulgaris and were found normal in none of the healthy controls.
A mild reduction of pulmonary diffusing capacity was observed in patients with pemphigus vulgaris.
寻常型天疱疮属于一组罕见的自身免疫性水疱性疾病,需要强化免疫抑制治疗。肺部感染在寻常型天疱疮患者中很常见。
评估寻常型天疱疮患者的肺功能,以检测可能使寻常型天疱疮患者易发生肺损伤的因素。
本研究纳入了10例初诊为重度寻常型天疱疮的患者和10名健康个体。进行了肺功能测试、血气分析和α1-抗胰蛋白酶血清水平定量检测。
与预测的正常平均值相比,记录到一氧化碳弥散能力轻度降低。寻常型天疱疮患者与健康对照者的单次呼吸一氧化碳弥散量(DLCOSB)值无显著差异,而一氧化碳弥散量与肺泡容积比值(DLCO/VA,VA:肺泡容积)的差异具有统计学意义。60%(6/10)的寻常型天疱疮患者α1-抗胰蛋白酶血清水平降低(<2.0g/L),而健康对照者中无一例正常。
寻常型天疱疮患者观察到肺弥散能力轻度降低。