Gündüz O H, Fertig N, Lucas M, Medsger T A
Ankara Numune Education and Research Hospital, Canyaka-Ankara, Turkey.
Arthritis Rheum. 2001 Jul;44(7):1663-6. doi: 10.1002/1529-0131(200107)44:7<1663::AID-ART290>3.0.CO;2-C.
To describe a series of systemic sclerosis (SSc) patients with the unusual combination of scleroderma renal crisis (SRC) and pulmonary hypertension (PHT) without interstitial lung disease.
The medical records of 2,459 SSc patients in the University of Pittsburgh Scleroderma Databank first evaluated between 1972 and 1999 were reviewed.
Eleven patients (0.45%) had both SRC and PHT. All had been evaluated since 1979, when angiotensin-converting enzyme (ACE) inhibitor therapy for SRC became available. Seven had SSc with limited cutaneous involvement, and 4 had SSc with diffuse cutaneous involvement. SRC occurred first in all patients except 1, in whom the onsets of SRC and PHT were simultaneous. SRC preceded PHT by a mean of 4.3 years. Four patients had anti-Th/To antibody, 5 had anti-RNA polymerase III antibody, 2 had anti-U3 RNP antibody, and none had anticentromere or antitopoisomerase I antibody. Ten of the 11 patients died, 8 from PHT. Ten patients were being treated with ACE inhibitor drugs when PHT developed.
In SSc, SRC and PHT are not mutually exclusive complications. SSc patients surviving SRC who have serum antibodies to Th/To, RNA polymerase III, or U3 RNP are at increased risk to develop PHT. ACE inhibitor therapy did not prevent the development of PHT.
描述一系列系统性硬化症（SSc）患者，这些患者患有硬皮病肾危象（SRC）和肺动脉高压（PHT）的不寻常组合，且无间质性肺病。
回顾了匹兹堡大学硬皮病数据库中1972年至1999年间首次评估的2459例SSc患者的病历。
11例患者（0.45%）同时患有SRC和PHT。自1979年有用于SRC的血管紧张素转换酶（ACE）抑制剂治疗以来，所有患者均接受了评估。7例为局限性皮肤受累的SSc，4例为弥漫性皮肤受累的SSc。除1例患者SRC和PHT同时发病外，所有患者均先出现SRC。SRC比PHT平均早4.3年出现。4例患者有抗Th/To抗体，5例有抗RNA聚合酶III抗体，2例有抗U3 RNP抗体，无一例有抗着丝点或抗拓扑异构酶I抗体。11例患者中有10例死亡，8例死于PHT。10例患者在PHT发生时正在接受ACE抑制剂药物治疗。
在SSc中，SRC和PHT并非相互排斥的并发症。在SRC后存活且有抗Th/To、RNA聚合酶III或U3 RNP血清抗体的SSc患者发生PHT的风险增加。ACE抑制剂治疗不能预防PHT的发生。
