Sanchez-Burson Juan, Garcia-Porrua Carlos, Montero-Granados Rafael, Gonzalez-Escribano Francisca, Gonzalez-Gay Miguel A
Division of Rheumatology, Valme University Hospital, Seville, Spain.
Semin Arthritis Rheum. 2002 Oct;32(2):125-9. doi: 10.1053/sarh.2002.33718.
To examine immunogenetic and clinical features in a series of patients with the idiopathic tubulointerstitial nephritis and uveitis (TINU) syndrome diagnosed at the single referral hospital for a defined population in Southern Spain.
Retrospective study of the case records of all patients diagnosed with the TINU syndrome in the Departments of Ophthalmology and Medicine of the Valme University Hospital (Seville, Spain) from January 1996 through October 2000. Patients were included in this study if they had a renal biopsy showing interstitial edema and infiltration by lymphocytes, plasma cells, macrophages, eosinophils, and neutrophils. In these cases fibrosis was occasionally seen, but no glomerular changes were found. In addition, a diagnosis of uveitis by expert ophthalmologists was always required. Underlying diseases, which might be responsible for the renal or ocular manifestations, were excluded. Patients were HLA-DRB1 genotyped from DNA by using molecular-based methods.
Six patients (4 females) fulfilled the definitions described above. Four were younger than 18 years. In addition to tubulointerstitial nephritis, non-granulomatous uveitis (anterior or panuveitis) associated with low visual acuity was present at the time of diagnosis. Leukocytosis and increase of acute phase reactants were also commonly observed at the time of diagnosis. Topical and oral corticosteroids were prescribed to all the patients. Cyclosporine A therapy was required in 2 cases. After a 2.5-year median follow-up, visual acuity had improved in all cases. Of note, 4 of 6 patients carried the HLA-DRB1*01 allele.
The TINU syndrome should be considered in the differential diagnosis of patients presenting with visual and renal manifestations. The presence of renal dysfunction in patients with uveitis may be of some help, as a warning sign, for the recognition of patients who require a rapid diagnosis and therapy. In Southern Spain, the TINU syndrome appears to be associated with HLA-DRB1*01 allele.
在西班牙南部一家指定的转诊医院，对一系列被诊断为特发性肾小管间质性肾炎和葡萄膜炎（TINU）综合征的患者进行免疫遗传学和临床特征研究。
对1996年1月至2000年10月期间在西班牙塞维利亚瓦尔梅大学医院眼科和内科被诊断为TINU综合征的所有患者的病历进行回顾性研究。如果患者的肾活检显示间质水肿以及淋巴细胞、浆细胞、巨噬细胞、嗜酸性粒细胞和中性粒细胞浸润，则纳入本研究。在这些病例中偶尔可见纤维化，但未发现肾小球病变。此外，始终需要由专业眼科医生做出葡萄膜炎的诊断。排除可能导致肾脏或眼部表现的基础疾病。采用基于分子的方法对患者的DNA进行HLA - DRB1基因分型。
6例患者（4例女性）符合上述定义。4例年龄小于18岁。除肾小管间质性肾炎外，诊断时还存在与低视力相关的非肉芽肿性葡萄膜炎（前葡萄膜炎或全葡萄膜炎）。诊断时还常见白细胞增多和急性期反应物增加。所有患者均接受了局部和口服皮质类固醇治疗。2例患者需要环孢素A治疗。经过2.5年的中位随访，所有病例的视力均有改善。值得注意的是，6例患者中有4例携带HLA - DRB1*01等位基因。
在鉴别诊断有视力和肾脏表现的患者时应考虑TINU综合征。葡萄膜炎患者出现肾功能障碍可能有助于作为一个警示信号，识别需要快速诊断和治疗的患者。在西班牙南部，TINU综合征似乎与HLA - DRB1*01等位基因相关。
