DeMarco Paul J, Weisman Michael H, Seibold James R, Furst Daniel E, Wong Weng Kee, Hurwitz Eric L, Mayes Maureen, White Barbara, Wigley Fredrick, Barr Walter, Moreland Larry, Medsger Thomas A, Steen Virginia, Martin Richard W, Collier David, Weinstein Arthur, Lally Edward, Varga John, Weiner Steven R, Andrews Brian, Abeles Micha, Clements Philip J
Marshfield Clinic, Wausau, Wisconsin, USA.
Arthritis Rheum. 2002 Nov;46(11):2983-9. doi: 10.1002/art.10589.
The reported frequency of scleroderma M01-R renal crisis (SRC) in diffuse systemic sclerosis (SSc; scleroderma) is 15-20%. Early use of angiotensin-converting enzyme (ACE) inhibitors has markedly improved outcome. The present analysis reexamines the prognostic factors for and outcome of SRC in a prospective cohort of patients with early diffuse SSc.
We retrospectively evaluated the cohort of SSc patients who participated in the High-Dose Versus Low-Dose D-Penicillamine in Early Diffuse SSc trial. Patients with diffuse cutaneous scleroderma were enrolled if their disease duration was <18 months. Because the trial failed to show a difference between treatment groups, the data were pooled.
One hundred thirty-four SSc patients entered the observation period a mean +/- SD of 0.8 +/- 0.3 years after onset of SSc. SRC occurred in 18 patients a mean +/- SD of 0.9 +/- 1.1 years after entry. During a mean +/- SD 4.0 +/- 1.1 years of followup after entry, 9 of the 18 patients died (mean +/- SD 0.6 +/- 0.9 years after SRC onset). Baseline characteristics that predicted SRC included a modified Rodnan skin thickness score of >or=20 (P < 0.01), enlarged cardiac silhouette on radiograph (P = 0.04), large joint contractures (wrist, elbow, knee) (P = 0.008), and prednisone use at entry (P = 0.01). Baseline characteristics that did not predict SRC included age, sex, race, Health Assessment Questionnaire score, fist closure, handspread, lung involvement, muscle weakness, erythrocyte sedimentation rate, and platelet count. In 5 of 10 subjects for whom at least 2 sequential skin scores were available, skin scores increased significantly (P = 0.012) in the 6 months before onset of SRC.
SRC occurred in 13% of patients soon (mean 11 months) after entry into the cohort. Predictors of SRC identified in this study included higher than average skin score, prednisone use at study entry, large joint contractures, and heart enlargement. Our data suggest, however, that low-dose prednisone alone was not associated with the onset of SRC, except in the appropriate clinical setting. Although ACE inhibitors and dialysis are now readily available, SRC continues to be associated with poor survival (in this study, 50% of patients with SRC died).
据报道，弥漫性系统性硬化症（SSc；硬皮病）中硬皮病M01-R肾危象（SRC）的发生率为15%-20%。早期使用血管紧张素转换酶（ACE）抑制剂显著改善了预后。本分析重新审视了早期弥漫性SSc患者前瞻性队列中SRC的预后因素和结局。
我们回顾性评估了参与早期弥漫性SSc高剂量与低剂量D-青霉胺试验的SSc患者队列。如果弥漫性皮肤硬皮病患者的病程<18个月，则纳入研究。由于该试验未显示治疗组之间存在差异，因此将数据合并。
134例SSc患者进入观察期，SSc发病后平均±标准差为0.8±0.3年。18例患者发生SRC，入组后平均±标准差为0.9±1.1年。入组后平均±标准差4.0±1.1年的随访期间，18例患者中有9例死亡（SRC发病后平均±标准差0.6±0.9年）。预测SRC的基线特征包括改良Rodnan皮肤厚度评分≥20（P<0.01）、X线片显示心脏轮廓增大（P=0.04）、大关节挛缩（腕、肘、膝）（P=0.008）以及入组时使用泼尼松（P=0.01）。未预测SRC的基线特征包括年龄、性别、种族、健康评估问卷评分、握拳、手伸展度、肺部受累、肌肉无力、红细胞沉降率和血小板计数。在10名至少有2次连续皮肤评分的受试者中，有5名在SRC发病前6个月皮肤评分显著增加（P=0.012）。
入组后不久（平均11个月），13%的患者发生SRC。本研究中确定的SRC预测因素包括高于平均水平的皮肤评分、研究入组时使用泼尼松、大关节挛缩和心脏扩大。然而，我们的数据表明，单独使用低剂量泼尼松与SRC的发生无关，除非在适当的临床环境中。尽管现在很容易获得ACE抑制剂和透析治疗，但SRC仍然与生存率低相关（在本研究中，50%的SRC患者死亡）。
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