特发性腹膜后纤维化：临床特征、治疗及结局的回顾性研究。
Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes.
机构信息
Division of Rheumatology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
出版信息
Mayo Clin Proc. 2011 Apr;86(4):297-303. doi: 10.4065/mcp.2010.0663.
OBJECTIVE
To describe the clinical manifestations, laboratory results, imaging findings, and treatments in patients with idiopathic retroperitoneal fibrosis (IRF) seen at Mayo Clinic in Rochester, MN.
PATIENTS AND METHODS
In this retrospective study, we used International Classification of Diseases, Ninth Revision codes to identify all patients evaluated for IRF between January 1, 1996, and December 31, 2006, at Mayo Clinic in Rochester, MN. Medical records were reviewed, and clinical information was abstracted. Idiopathic retroperitoneal fibrosis was diagnosed on the basis of compatible imaging findings. Patients were followed up until their last visit at Mayo Clinic, death, or December 31, 2008, whichever came first.
RESULTS
Of the 185 patients identified as having IRF, 113 (61%) were men and 72 (39%) were women. Mean ± SD age at diagnosis was 57.6 ± 11.8 years. Biopsy specimens were obtained in 142 cases (77%). The most common presenting symptoms were back pain (38%) and abdominal pain (40%). Baseline erythrocyte sedimentation rate and/or C-reactive protein levels were elevated in 88 (58%) of the 151 patients tested. The median creatinine level at diagnosis was 1.3 mg/dL (interquartile range, 1.1-2.1 mg/dL). Fifteen patients (8%) were treated with ureteral procedures only, 58 patients (31%) with medications only, and 105 patients (57%) with a combination of medical and surgical therapies. Seven patients (4%) were not treated. Corticosteroids were initiated in 116 patients (63%), and tamoxifen was used in 120 patients (65%). Follow-up was available for 151 patients (82%). Creatinine levels were normal at last visit in 102 (68%) of the 151 patients with follow-up. No patient developed end-stage renal disease. Relapses occurred in 18 (12%) of the 151 patients. Eleven patients died.
CONCLUSION
In this cohort, outcomes such as end-stage renal disease or death from renal failure were not observed. Relapses may occur, and patients with IRF warrant long-term follow-up.
目的
描述在明尼苏达州罗切斯特的梅奥诊所就诊的特发性腹膜后纤维化（IRF）患者的临床表现、实验室结果、影像学表现和治疗方法。
患者和方法
在这项回顾性研究中，我们使用国际疾病分类，第九版代码来确定 1996 年 1 月 1 日至 2006 年 12 月 31 日期间在明尼苏达州罗切斯特的梅奥诊所就诊的所有特发性腹膜后纤维化患者。我们对病历进行了审查，并提取了临床信息。特发性腹膜后纤维化是根据相容的影像学表现诊断的。我们对患者进行了随访，直到他们在梅奥诊所的最后一次就诊、死亡或 2008 年 12 月 31 日（以先到者为准）。
结果
在确定的 185 例特发性腹膜后纤维化患者中，113 例（61%）为男性，72 例（39%）为女性。诊断时的平均年龄 ± SD 为 57.6 ± 11.8 岁。142 例（77%）患者进行了活检。最常见的首发症状是背痛（38%）和腹痛（40%）。在 151 例接受检测的患者中，88 例（58%）的红细胞沉降率和/或 C 反应蛋白水平升高。诊断时中位数的血肌酐水平为 1.3 mg/dL（四分位距，1.1-2.1 mg/dL）。15 例（8%）患者仅接受了输尿管手术治疗，58 例（31%）患者仅接受了药物治疗，105 例（57%）患者接受了药物和手术联合治疗。7 例（4%）患者未接受治疗。116 例（63%）患者开始使用皮质类固醇，120 例（65%）患者使用他莫昔芬。对 151 例（82%）患者进行了随访。在有随访的 151 例患者中，最后一次就诊时 102 例（68%）的血肌酐水平正常。没有患者发展为终末期肾病。151 例患者中有 18 例（12%）复发。11 例患者死亡。
结论
在该队列中，没有观察到终末期肾病或肾衰竭导致的死亡等结局。可能会出现复发，因此特发性腹膜后纤维化患者需要长期随访。
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