Konma Junichi, Kotani Takuya, Shoda Takeshi, Suzuka Takayasu, Fujiki Youhei, Nagai Koji, Hata Kenichiro, Yoshida Shuzo, Takeuchi Tohru, Makino Shigeki, Arawaka Shigeki
a Department of Internal Medicine (IV) , Osaka Medical College , Takatsuki , Osaka , Japan.
Mod Rheumatol. 2018 Nov;28(6):1009-1015. doi: 10.1080/14397595.2018.1441658. Epub 2018 Mar 2.
We retrospectively investigated efficacy and safety of combination therapy with prednisolone (PSL) and tacrolimus (TAC) for progressive interstitial pneumonitis with systemic sclerosis (SSc-PIP).
We studied 11 patients with SSc-PIP who received combination therapy with PSL (0.5 mg/kg/d) and TAC (3 mg/d).
Baseline Hugh-Jones grades were I, II, III, and IV in 2, 6, 2, and 1 patients, respectively. Krebs von den Lungen-6 (KL-6) values were elevated to 914 (range 300-2614) U/mL. % Diffusing capacity of carbon monoxide (%DLco) remarkably decreased to 47.4 (range 9.7-64.4) %. All patients were alive at 1 year after therapy. In response to treatment, interstitial pneumonia (IP) improved in three patients, stable in seven patients, and deteriorated in one patient. Total ground-glass opacity (GGO) score improved (p = .005). No significant changes occurred in values of KL-6, % forced vital capacity (%FVC), and %DLco. Presently, all seven patients who could be followed up were alive. IP improved in three patients and stable in four patients. Total GGO score improved (p = .016). KL-6, %FVC, and %DLco did not change. Mild cytomegalovirus or herpes zoster infection occurred in two patients. Grade I renal injuries were observed in three and one patient at 1 year and present, respectively.
Combination therapy with PSL and TAC appeared to be well tolerated and effective in suppressing the disease activity of SSc-PIP.
我们回顾性研究了泼尼松龙（PSL）和他克莫司（TAC）联合治疗系统性硬化症相关进行性间质性肺炎（SSc-PIP）的疗效和安全性。
我们研究了11例接受PSL（0.5mg/kg/d）和TAC（3mg/d）联合治疗的SSc-PIP患者。
基线时，Hugh-Jones分级为I级、II级、III级和IV级的患者分别有2例、6例、2例和1例。克雷伯斯-冯-登-卢肯斯-6（KL-6）值升高至914（范围300-2614）U/mL。一氧化碳弥散量百分比（%DLco）显著降至47.4（范围9.7-64.4）%。所有患者在治疗后1年时均存活。治疗后，3例间质性肺炎（IP）患者病情改善，7例稳定，1例恶化。总磨玻璃影（GGO）评分改善（p = 0.005）。KL-6、用力肺活量百分比（%FVC）和%DLco值无显著变化。目前，所有7例可随访的患者均存活。3例IP患者病情改善，4例稳定。总GGO评分改善（p = 0.016）。KL-6、%FVC和%DLco未改变。2例患者发生轻度巨细胞病毒或带状疱疹感染。分别有3例和1例患者在1年时及目前观察到I级肾损伤。
PSL和TAC联合治疗似乎耐受性良好，且在抑制SSc-PIP疾病活动方面有效。
