Akpolat Tekin, Dilek Melda, Aksu Kenan, Keser Gökhan, Toprak Omer, Cirit Mustafa, Oğuz Yusuf, Taşkapan Hülya, Adibelli Zelal, Akar Harun, Tokgöz Bülent, Arici Mustafa, Celiker Hüseyin, Diri Banu, Akpolat Ilkser
Ondokuz Mayis University School of Medicine, Samsun, Turkey.
Semin Arthritis Rheum. 2008 Dec;38(3):241-8. doi: 10.1016/j.semarthrit.2007.11.001. Epub 2008 Jan 25.
The aims of this study are (1) to report 33 patients with Behçet's disease (BD) having various renal manifestations, and (2) to update current data using our patients and published papers about BD and renal manifestations.
The PubMed database was searched using the terms BD or Behçet's syndrome. We found reports of 94 patients (including ours) with BD and specific renal diseases (amyloidosis, 39; glomerulonephritis [GN], 37; renal vascular disease, 19; interstitial nephritis, 1).
The presentation of renal disease was edema/nephrotic syndrome in 12 patients (36%). Renal disease was incidentally diagnosed by routine urine analysis and measurement of serum creatinine level in 20 patients (61%). Renal failure was present in 23 patients (70%) and 5 of them have had cyclosporine treatment. The frequency of renal disease among BD patients has been reported to vary from less than 1 to 29%.
The clinical spectrum of renal BD shows a wide variation. Amyloidosis (AA type), GN, and macroscopic/microscopic vascular disease are the main causes of renal BD. Patients with vascular involvement have a high risk of amyloidosis and amyloidosis is the most common cause of renal failure in BD. Several types of glomerular lesions are seen in BD. Current treatment options for renal BD are not evidence based. Radiological vascular intervention combined with immunosuppressive drugs can be useful in selected cases. Routine urine analysis and measurement of serum creatinine level are needed for early diagnosis of renal BD.
本研究的目的是（1）报告33例患有各种肾脏表现的白塞病（BD）患者，以及（2）利用我们的患者和已发表的关于BD与肾脏表现的论文更新当前数据。
使用术语BD或白塞综合征在PubMed数据库中进行检索。我们发现了94例（包括我们的患者）患有BD和特定肾脏疾病（淀粉样变性，39例；肾小球肾炎[GN]，37例；肾血管疾病，19例；间质性肾炎，1例）的患者报告。
12例患者（36%）的肾脏疾病表现为水肿/肾病综合征。20例患者（61%）通过常规尿液分析和血清肌酐水平测量偶然诊断出肾脏疾病。23例患者（70%）存在肾衰竭，其中5例接受了环孢素治疗。据报道，BD患者中肾脏疾病的发生率在不到1%至29%之间变化。
肾脏BD的临床谱表现出广泛差异。淀粉样变性（AA型）、GN和宏观/微观血管疾病是肾脏BD的主要原因。血管受累的患者发生淀粉样变性的风险很高，淀粉样变性是BD患者肾衰竭的最常见原因。BD中可见几种类型的肾小球病变。目前肾脏BD的治疗选择缺乏循证依据。放射学血管介入联合免疫抑制药物在某些病例中可能有用。肾脏BD的早期诊断需要常规尿液分析和血清肌酐水平测量。
