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摘要

我们可以在3%的肾活检中发现小管间质性肾炎，当出现急性肾衰竭时约为12-15%。小管间质性肾炎和葡萄膜炎(TINU综合征)是一种罕见的情况，很少有文献报道的病例。通常表现为合并肾衰竭和葡萄膜炎，经肾活检诊断，并诊断排除。我们描述了一例综合征TINU诊断的肾活检结果特征为显性t淋巴细胞和肥大细胞，对皮质类固醇治疗有良好的反应。

关键字

小管间质性肾炎，葡萄膜炎，t淋巴细胞疾病

病例报告

第一例病例描述于1975年。TINU综合征是一种非常罕见的疾病;文献报道了超过250例[1]。TINU综合征是小管间质性肾炎和复发性葡萄膜炎的结合。大多数病例已在眼科和儿科杂志上报道。

一名37岁男性因肌酐升高(258.13 mmol/L)由他的初级保健医生推荐。过去四年有葡萄膜炎病史，无具体病因，接受强的松治疗;然而，由于完全缓解，他6个月没有接受任何治疗。无排尿困难、血尿、少尿或其他泌尿系统症状。他否认自己经常服用任何药物。眼科检查发现左前葡萄膜炎;其余的身体检查都很正常。实验室检查示轻度正常细胞性贫血伴11.9%嗜酸性粒细胞增多，血清肌酐282.8 mmol / L(参考文献35.4-97.2)，抗体IgG阳性。尿分析显示蛋白尿1500 mg/24小时，无红细胞增多。其他检查，包括血清补体、IgG、IgG亚类、IgM、类风湿因子、抗核抗体、抗双链dna抗体、抗ss - a /SS-B抗体、抗中性粒细胞胞浆抗体、HLA - b27、血管紧张素转换酶(ACE)水平、血/尿培养、粪便分析、粪便培养、抗梅毒螺旋体、曲霉、半乳甘露聚糖抗原、棘球蚴、布鲁氏菌、刚地弓形虫抗体、HIV血清学、曼妥氏试验和乙肝/丙肝。 showed negative / normal results. The Chest X -Ray was normal. The Renal ultrasound revealed increased echogenicity and decreased corticomedullary ratio. Renal biopsy was performed by analyzing 22 glomeruli, 11 of them sclerotic, with moderate tubular atrophy, and interstitial fibrosis. The interstitial inflammation was multifocal and mixed type, with no signs of vasculitis and absence of granulomas. The study of immunofluorescence for IgA, IgG, IgM, C3, fibrinogen, C1q and light chains (K/L) were negative. The Immunophenotyping of interstitial inflammatory infiltrate highlighted a higher proportion of T lymphocytes (CD3) on B lymphocytes (CD20), with a low proportion of plasma and macrophage cells, as well as accumulation of mast cells in areas of greater chronicity. We finally diagnose tubulointerstitial nephritis and uveitis (TINU syndrome); we started treatment with intravenous methylprednisolone 250 mg and topic corticosteroids each day, for three consecutive days and then oral prednisone 1 mg/Kg/day. Our patient, one month later improved with a serum creatinine of 221 mmol/L. Evidence suggests that modified C- reactive protein (mCRP), an autoantigen present in the renal tubular cells, and uvea are associated in its pathogenesis [2]. Delayed-type hypersensitivity and suppressed cell-mediated immunity with a predominance of T-lymphocytes and mast cells likely play a significant role as our case demonstrated. Renal tubulointerstitial infiltrates are primarily composed of activated T- lymphocytes [3,4]. Has predominance in adolescence and is more common around the 15 years old. There have been identified up to 50% of cases as triggers the use of NSAIDs, chinese herbs (goreisan, hypoparathyroidism, hyperthyroidism, IgG4 - related disease, concurrent chlamydial infection and Epstein – Barr virus. The main clinical manifestations are fever, weight loss, abdominal pain, arthralgias, myalgias and may be associated with uveitis. Renal manifestations include abdominal pain mainly on flanks, sterile pyuria, polyuria, nocturia, hematuria and proteinuria in subnephrotic range. Laboratory findings can reveal anemia, elevated erythrocyte sedimentation rate, eosinophilia, presence of cytoplasmic antineutrophil antibody (ANCA) [5], rheumatoid factor or hypocomplementemia. TINU syndrome diagnosis is nonspecific and is made by the combination of uveitis and renal involvement, with renal biopsy consistent with acute tubulointerstitial nephritis. Typical findings include tubulointerstitial edema and inflammatory infiltration, cells composed mostly of mononuclear cells, such as T-lymphocytes, mast cells, plasma cells and histiocytes. Findings with immunofluorescence and electron microscopy are nonspecific. The differential diagnosis includes sarcoidosis, sjögrens´s syndrome, systemic lupus erythematosus, Behcet´s disease, toxoplasmosis, histoplasmosis, tuberculosis. Prognosis is favorable with full recovery in 80% of cases and depends on the degree of tubulointerstitial fibrosis. Patients with progressive renal failure are treated with prednisone 1 mg / kg for three to six months and then slowly tapered. Uveitis has a favorable response due to the use of topical and systemic corticosteroids. Hausleret al。[6]最近报道了一例对皮质激素和环孢素不耐的TINU综合征，在阿达木单抗联合甲氨蝶呤治疗后有良好的结果(图1)。

图1所示。(A)肾活检显示中度间质炎症、小管萎缩和间质纤维化(苏木精和伊红，原始放大x 40;插入:马松三色，原始放大倍数× 100)。(B) T淋巴细胞比例高(CD3免疫染色，原放大倍数x100)。(C) B淋巴细胞比例减少(CD20免疫染色，原放大倍数x 100)。(D)巨噬细胞比例低(CD68免疫染色，原始放大倍数x 100)。(E)肥大细胞在较慢性病变区域的聚集(CD117免疫染色，原始放大倍数x 40)。

参考文献

2021年版权燕麦。所有权利reserv
1. 李晨，苏婷，褚锐，李旭，杨玲(2014)中国成人小管间质性肾炎合并葡萄膜炎。临床J Am Soc肾小球9:第21至28。(Crossref)
2. 田中平，田中平，田中平，田中平，等。(2001)1例小管间质性肾炎和葡萄膜炎综合征女孩的重复肾活检。Pediatr Nephrol11: 885 - 887。(Crossref)
3. Yano N, Miyazaki M, Endoh M, Kuramoto T, Eguchi K，等。(1991)特发性急性间质性肾炎合并葡萄膜炎。日本J地中海30: 548 - 552。(Crossref)
4. 谭涛，于芳，曲铮，苏涛，邢国强等(2011)修饰的c反应蛋白可能是TINU综合征的靶向自身抗原。临床J Am Soc肾小球2011;6: 93。(Crossref)
5. Simon AH, Alves-Filho G, ribeio - alves MA(1996)急性小管间质性肾炎和葡萄膜炎伴抗中性粒细胞胞浆抗体。肾病28: 124。(Crossref)
6. Häusler U, Guminski B, Helmchen U，等(2013)小管间质性肾炎伴葡萄膜炎(TINU)综合征。一个相对罕见的风湿病鉴别诊断为不明原因葡萄膜炎。Z Rheumatol72: 393 - 7。(Crossref)