Sacks D G, Okano Y, Steen V D, Curtiss E, Shapiro L S, Medsger T A
Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, PA, USA.
J Rheumatol. 1996 Apr;23(4):639-42.
To describe a group of patients with systemic sclerosis (SSc) and diffuse cutaneous (dc) involvement with isolated pulmonary hypertension (IPHT) and to compare them to 2 other SSc patient groups, i.e., one with limited cutaneous (lc) involvement with IPHT and another with dcSSc without IPHT.
The Pittsburgh Scleroderma Databank was screened to identify appropriate patients. SSc specific serum autoantibodies were determined using published methods. Chi-squared tests and Kaplan-Meier survival analysis were performed.
During the period 1975-92, 60 of 580 (10%) consecutive new patients with lcSSc and 14 of 677 (2%) with dcSSc have developed IPHT (p < 0.0001). The patients with IPHT did not differ from their parent dc or lc groups by age, race, sex, or cutaneous manifestations. All 14 patients with dcSSc IPHT had minimal or no pulmonary interstitial fibrosis on chest radiograph. The 12 tested had a striking reduction in DLCO (mean 50% of predicted normal), similar to results for patients with lcSSc with IPHT. Of note, 5 of these 14 patients had survived "scleroderma renal crisis" 1.3 to 10.7 (mean 5.5) years before onset of IPHT. Six of 13 (46%) patients with dcSSc with IPHT were positive for serum anti-U3RNP antibody by immunoprecipitation, and one had the typical nucleolar staining pattern of anti-U3RNP, but was not tested for U3RNP by immunoprecipitation. In contrast, only 13 (6%) of the 244 patients with dcSSc without IPHT (p < 0.0001) had anti-U3RNP antibody. Survival from the first reported symptom consistent with pulmonary hypertension (usually dyspnea on exertion) was uniformly poor in both IPHT groups, with the vast majority of patients dying during the first 2 years.
Patients with SSc with dc involvement can develop severe, fatal isolated pulmonary arterial hypertension. This complication occurs disproportionately more often in patients with serum anti-U3RNP antibody.
描述一组系统性硬化症（SSc）合并弥漫性皮肤受累及孤立性肺动脉高压（IPHT）的患者，并将其与另外两组SSc患者进行比较，即一组为局限性皮肤受累合并IPHT的患者，另一组为无IPHT的弥漫性皮肤型SSc（dcSSc）患者。
对匹兹堡硬皮病数据库进行筛查以确定合适的患者。采用已发表的方法测定SSc特异性血清自身抗体。进行卡方检验和Kaplan-Meier生存分析。
在1975年至1992年期间，580例连续的新发局限性皮肤型SSc患者中有60例（10%）、677例dcSSc患者中有14例（2%）发生了IPHT（p<0.0001）。IPHT患者在年龄、种族、性别或皮肤表现方面与其母组dc或lc组无差异。所有14例dcSSc合并IPHT的患者胸部X线片上肺间质纤维化轻微或无。12例接受检测的患者的一氧化碳弥散量（DLCO）显著降低（平均为预测正常值的50%），与局限性皮肤型SSc合并IPHT的患者结果相似。值得注意的是，这14例患者中有5例在IPHT发病前1.3至10.7年（平均5.5年）曾经历“硬皮病肾危象”并存活下来。13例dcSSc合并IPHT的患者中有6例（46%）免疫沉淀法检测血清抗U3RNP抗体呈阳性，1例具有抗U3RNP典型的核仁染色模式，但未通过免疫沉淀法检测U3RNP。相比之下，244例无IPHT的dcSSc患者中只有13例（6%）有抗U3RNP抗体（p<0.0001）。在两个IPHT组中，从首次出现与肺动脉高压相符的症状（通常为劳力性呼吸困难）开始的生存率均较差，绝大多数患者在最初2年内死亡。
SSc合并dc受累的患者可发生严重的、致命性孤立性肺动脉高压。这种并发症在血清抗U3RNP抗体阳性的患者中发生比例更高。
