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

机译:肺结核和常见可变免疫缺陷患者的继发性肾淀粉样变性

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摘要

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.
机译:常见的可变免疫缺陷症(CVID)通常表现在生命的第二个或第三个十年中,并伴有反复的细菌感染和血球蛋白不足。尽管很少有CVID患者报告有T细胞功能障碍,但在CVID中很少有继发性肾淀粉样变性病伴有肺结核病史。一名40岁男性因3个月的反复呼吸道感染和持续性进站脚趾浮肿而入院。他的过去历史显示,自最近20年以来,每年有3至5次反复发作的呼吸道感染和腹泻。 8年前,他已经成功治疗了痰阳性的肺结核。实验室研究显示高红细胞沉降率(ESR),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)阳性继发性肾脏淀粉样变性。肾小球肾小球系膜区变宽,伴有周期性席夫氏染色(PAS)沉积,呈粉红色基质,呈浅阳性,在刚果红染色上呈苹果绿色双折射。免疫组织化学为AA染色阳性。免疫荧光显微镜检查未发现抗人IgG,IgM,IgA,C3,C1q,κ和λ轻链抗血清染色。对症患者接受了呼吸道感染的对症治疗,并用低剂量血管紧张素受体阻滞剂出院。人们认为,经过治疗的旧结核病和由于反复呼吸道感染引起的慢性炎症是造成AA淀粉样变性的原因。因此,在CVID患者的AA肾淀粉样变性的继发原因的鉴别诊断中应考虑肺结核,特别是在地方性环境中。

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