Ounissi Mondher, Ben Abdelghani Khaoula, Gargueh Taher, Turki Sami, Goucha Rym, Abderrahim Ezzedine, Ben Hamida Fathi, Ben Maiz Hedi, Khedher Adel
Service de Médecine interne A - Hôpital Charles Nicolle de Tunis.
Tunis Med. 2010 Jun;88(6):404-8.
Renal involvement in the Behcet's disease is rare. The clinical features vary from urinary sediment's abnormalities to ESRD.
We propose to study the clinical, biological and histological data, the therapeutic management and the prognosis of patients.
We report a retrospective study including 8 patients representing 1.23 % of cases.
The average age of the patient was of 37 +/- 12. 35 years with a clear male prevalence. Urinary signs were discovered fortuitously by the strips in the majority of the cases after an average of 18 months. It's about proteinuria and hematuria. Renal insufficiency and hypertension were rare. Pathological study highlighted 3 cases of amyloidosis, 2 cases of IgA nephropathy, 1 case of minimal change disease, 1 case of endo and extracapillary glomerulonephritis and 1 case of interstitial nephropathy. Patients having GN were treated by corticoids and immunosuppressive agents and those having an interstitial nephropathy were treated symptomatically with good evolution in the majority of the cases. Only one patient is dead, he had amyloidosis. Prognosis depended on the precocity of the diagnosis, the histological type and the treatment.
The renal involvement during Behçet's disease is rare. Amyloidosis and Ig A nephropathy are the most frequent. Treatment is still controversial.
白塞病累及肾脏较为罕见。临床特征从尿沉渣异常到终末期肾病各不相同。
我们建议研究患者的临床、生物学和组织学数据、治疗管理及预后。
我们报告一项回顾性研究，纳入8例患者，占病例总数的1.23%。
患者平均年龄为37±12.35岁，男性明显居多。多数病例中，尿异常表现是通过试纸偶然发现的，平均间隔18个月。表现为蛋白尿和血尿。肾功能不全和高血压较为少见。病理研究显示3例淀粉样变性、2例IgA肾病、1例微小病变病、1例毛细血管内和毛细血管外肾小球肾炎以及1例间质性肾病。患有肾小球肾炎的患者接受皮质类固醇和免疫抑制剂治疗，间质性肾病患者进行对症治疗，多数病例病情进展良好。仅1例患者死亡，该患者患有淀粉样变性。预后取决于诊断的及时性、组织学类型和治疗情况。
白塞病累及肾脏较为罕见。淀粉样变性和IgA肾病最为常见。治疗仍存在争议。
