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一名肺结核合并普通可变免疫缺陷患者的继发性肾淀粉样变性。

Secondary renal amyloidosis in a patient of pulmonary tuberculosis and common variable immunodeficiency.

作者信息

Balwani Manish R, Kute Vivek B, Shah Pankaj R, Wakhare Pawan, Trivedi Hargovind L

机构信息

Department of Nephrology and Clinical Transplantation and Institute of Kidney Diseases and Research Center, Dr. HL Trivedi Institute of Transplantation Sciences (IKDRC-ITS), Ahmedabad, India.

出版信息

J Nephropharmacol. 2015 Jun 21;4(2):69-71. eCollection 2015.

Abstract

Common variable immunodeficiency (CVID) usually manifests in the second or third decade of life with recurrent bacterial infections and hypoglobulinemia. Secondary renal amyloidosis with history of pulmonary tuberculosis is rare in CVID, although T cell dysfunction has been reported in few CVID patients. A 40-year-old male was admitted to our hospital with a 3-month history of recurrent respiratory infections and persistent pitting pedal edema. His past history revealed 3 to 5 episodes of recurrent respiratory tract infections and diarrhoea each year since last 20 years. He had been successfully treated for sputum positive pulmonary tuberculosis 8 years back. Laboratory studies disclosed high erythrocyte sedimentation rate (ESR), hypoalbuminemia and nephrotic range proteinuria. Serum immunoglobulin levels were low. CD4/CD8 ratio and CD3 level was normal. C3 and C4 complement levels were normal. Biopsy revealed amyloid A (AA) positive secondary renal amyloidosis. Glomeruli showed variable widening of mesangial regions with deposition of periodic schiff stain (PAS) pale positive of pink matrix showing apple green birefringence on Congo-red staining. Immunohistochemistry was AA stain positive. Immunofluorescence microscopy revealed no staining with anti-human IgG, IgM, IgA, C3, C1q, kappa and lambda light chains antisera. Patient was treated symptomatically for respiratory tract infection and was discharged with low dose angiotensin receptor blocker. An old treated tuberculosis and chronic inflammation due to recurrent respiratory tract infections were thought to be responsible for AA amyloidosis. Thus pulmonary tuberculosis should be considered in differential diagnosis of secondary causes of AA renal amyloidosis in patients of CVID especially in endemic settings.

摘要

常见变异型免疫缺陷(CVID)通常在生命的第二个或第三个十年表现为反复细菌感染和低球蛋白血症。尽管在少数CVID患者中报告有T细胞功能障碍,但伴有肺结核病史的继发性肾淀粉样变性在CVID中很少见。一名40岁男性因反复呼吸道感染3个月病史和持续性足背凹陷性水肿入院。他的既往史显示,自过去20年以来,每年有3至5次反复呼吸道感染和腹泻发作。他8年前曾成功治疗痰涂片阳性的肺结核。实验室检查显示红细胞沉降率(ESR)升高、低白蛋白血症和肾病范围蛋白尿。血清免疫球蛋白水平低。CD4/CD8比值和CD3水平正常。C3和C4补体水平正常。活检显示淀粉样蛋白A(AA)阳性的继发性肾淀粉样变性。肾小球系膜区可见不同程度增宽,伴有周期性席夫染色(PAS)淡粉红色基质沉积,刚果红染色显示苹果绿双折射。免疫组化AA染色阳性。免疫荧光显微镜检查显示抗人IgG、IgM、IgA、C3、C1q、κ和λ轻链抗血清无染色。患者因呼吸道感染接受对症治疗,出院时服用低剂量血管紧张素受体阻滞剂。既往治疗过的肺结核和反复呼吸道感染引起的慢性炎症被认为是AA淀粉样变性的原因。因此,在CVID患者中,尤其是在地方病流行地区,AA肾淀粉样变性的继发性病因鉴别诊断中应考虑肺结核。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac2/5297488/9c746a0f73e0/npj-4-69-g001.jpg

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