Litwin Mieczysław, Michałkiewicz Jacek, Jarmuek Wioletta, Woźniewicz Bogdan, Grenda Ryszard, Iwanicka Katarzyna, Madaliński Kazimierz
Department of Nephrology and Kidney Transplantation, The Children's Memorial Health Institute, Warsaw, Poland.
Pediatr Nephrol. 2002 Aug;17(8):683-8. doi: 10.1007/s00467-002-0891-5. Epub 2002 Jun 7.
A 10-year-old boy was evaluated for fever, weight loss, uveitis, normocytic, normochromic anemia, renal insufficiency, and hypergammaglobulinemia of 8 weeks' duration. Infectious and neoplastic causes of fever were excluded. A renal biopsy performed in the 4th week of disease revealed diffuse plasmocytic interstitial nephritis. No treatment was prescribed and the patient was transferred to another hospital. Because clinical symptoms and renal insufficiency were still present, in the 8th week of disease a second biopsy was performed, which showed lympho-monocytic interstitial nephritis. At the same time, phenotypic analysis of peripheral blood mononuclear cells was carried out, revealing a significantly decreased number of CD3(+), CD4(+), and CD3(+)/CD8(+) cells, increased non-T CD3(-)/CD8(+) and CD56(+) NK cells, and decreased "naïve" (CD45RA(+)/CD4(+)) and memory (CD45RO(+)/CD8(+)) T lymphocytes. A 6-month course of oral prednisone was prescribed. Clinical symptoms and laboratory findings quickly returned to normal values. After 13 days of corticosteroid therapy, a second phenotypic analysis of peripheral blood mononuclear cells was performed, which revealed normalization of CD3(+), CD4(+), and CD3(+)/CD8(+) cells as well as proportions of non-T CD8(+) and CD56(+) NK lymphocytes, "naïve" and memory cells. This case shows spontaneous evolution of tubulointerstitial infiltrates from plasmacytic to lympho-monocytic, profound disturbances of the immunological system, and the beneficial effect of corticosteroids on both the clinical course and immunological disturbances.
一名10岁男孩因发热、体重减轻、葡萄膜炎、正细胞正色素性贫血、肾功能不全以及持续8周的高球蛋白血症接受评估。发热的感染性和肿瘤性病因被排除。在病程第4周进行的肾活检显示弥漫性浆细胞性间质性肾炎。未给予治疗，患者转至另一家医院。由于临床症状和肾功能不全仍然存在，在病程第8周进行了第二次活检，结果显示淋巴细胞 - 单核细胞性间质性肾炎。同时，对外周血单个核细胞进行了表型分析，结果显示CD3(+)、CD4(+)和CD3(+)/CD8(+)细胞数量显著减少，非T CD3(-)/CD8(+)和CD56(+) NK细胞增加，“幼稚”(CD45RA(+)/CD4(+))和记忆(CD45RO(+)/CD8(+)) T淋巴细胞减少。给予了为期6个月的口服泼尼松疗程。临床症状和实验室检查结果迅速恢复至正常水平。在皮质类固醇治疗13天后，对外周血单个核细胞进行了第二次表型分析，结果显示CD3(+)、CD4(+)和CD3(+)/CD8(+)细胞以及非T CD8(+)和CD56(+) NK淋巴细胞、“幼稚”和记忆细胞的比例恢复正常。该病例显示肾小管间质浸润从浆细胞性自发演变为淋巴细胞 - 单核细胞性，免疫系统存在严重紊乱，以及皮质类固醇对临床病程和免疫紊乱均有有益作用。
