Nawata Y, Kurasawa K, Takabayashi K, Miike S, Watanabe N, Hiraguri M, Kita Y, Kawai M, Saito Y, Iwamoto I
Department of Internal Medicine, Chiba University School of Medicine, Japan.
J Rheumatol. 1999 Jul;26(7):1527-33.
To determine the characteristics of corticosteroid resistant interstitial pneumonitis (IP) in dermatomyositis (DM) and polymyositis (PM), and to evaluate the effect of cyclosporine on corticosteroid resistant IP in DM/PM.
We analyzed retrospectively the incidence, clinical features, and corticosteroid responses of IP in 111 patients with DM (56) or PM (55). All patients with DM/PM were treated with prednisolone, and corticosteroid resistant IP was defined as a progression of IP despite administration of 1 mg/kg/day prednisolone for more than 4 weeks. We also evaluated the effect of cyclosporine on corticosteroid resistant IP in patients with DM/PM.
IP occurred in 24 of 56 DM and 12 of 55 PM patients. We then classified IP in DM/PM according to serum CPK levels at the onset of IP; IP associated with high CPK levels (type I) (19) and IP associated with normal CPK levels (type II) (17). Only 2 of 19 (11%) type I IP were resistant to prednisolone therapy, while 14 of 17 (82%) type II IP were resistant to prednisolone therapy. Thus, patients with type II IP showed poorer prognosis than those with type I IP (one year survival rate: type I 89% vs type II 31%). Cyclosporine was effective in all 5 cases with corticosteroid resistant IP in DM/PM (one year survival rate 80%).
(1) Corticosteroid resistant IP develops mostly in patients with DM/PM without CPK elevation at the onset of IP (type II IP), and (2) cyclosporine is effective for the corticosteroid resistant IP in DM/PM and significantly prolongs survival of patients.
确定皮肌炎（DM）和多发性肌炎（PM）中糖皮质激素抵抗性间质性肺炎（IP）的特征，并评估环孢素对DM/PM中糖皮质激素抵抗性IP的疗效。
我们回顾性分析了111例DM（56例）或PM（55例）患者中IP的发病率、临床特征及糖皮质激素反应。所有DM/PM患者均接受泼尼松龙治疗，糖皮质激素抵抗性IP定义为尽管给予1mg/（kg·天）泼尼松龙治疗超过4周，IP仍进展。我们还评估了环孢素对DM/PM患者中糖皮质激素抵抗性IP的疗效。
56例DM患者中有24例发生IP，55例PM患者中有12例发生IP。然后我们根据IP发病时的血清肌酸磷酸激酶（CPK）水平对DM/PM中的IP进行分类；与高CPK水平相关的IP（I型）（19例）和与正常CPK水平相关的IP（II型）（17例）。19例I型IP中只有2例（11%）对泼尼松龙治疗耐药，而17例II型IP中有14例（82%）对泼尼松龙治疗耐药。因此，II型IP患者的预后比I型IP患者差（一年生存率：I型89% vs II型31%）。环孢素对DM/PM中所有5例糖皮质激素抵抗性IP均有效（一年生存率80%）。
（1）糖皮质激素抵抗性IP主要发生在IP发病时无CPK升高的DM/PM患者中（II型IP），（2）环孢素对DM/PM中糖皮质激素抵抗性IP有效，并能显著延长患者的生存期。
