Uthgenannt D, Steinhoff J, Baretton G, Sack K
Klinik für Innere Medizin, Medizinischen Universität Lübeck.
Dtsch Med Wochenschr. 1991 Jun 14;116(24):935-8. doi: 10.1055/s-2008-1063700.
A 45-year-old Turk had a year ago noticed a submandibular and a retroauricular node-like swelling, about 2 cm in diameter each, firm and freely mobile. During the preceding two months he had polydipsia and polyuria. Recently he developed a nephrotic syndrome with lower-leg oedema and proteinuria (14 g albumin in 24-hour urine). The concentrations of IgE (250 IE/ml) and IgA (745 mg/dl) were raised, and there was eosinophilia of 14%. Renal needle biopsy revealed glomerulonephritis with minimal proliferation. Excision of part of the nodular tumour revealed histologically the typical signs of Kimura's disease (eosinophilic follicular lymphadenitis of the skin; subcutaneous angiolymphoid hyperplasia with eosinophilia). During treatment with prednisolone, 20 mg daily by mouth, the clinical and biochemical findings regressed within two weeks. But eight weeks later, after dose reduction to 10 mg daily, the nephrotic syndrome recurred so that the dosage had to be increased again to 20 mg prednisolone daily. On this treatment the patient has now been symptom-free for six months. This case demonstrates the unusual association of Kimura's disease with minimally proliferative glomerulonephritis.
一名45岁的土耳其人在一年前注意到下颌下和耳后有结节样肿胀,每个直径约2厘米,质地坚硬,可自由活动。在之前的两个月里,他出现了多饮和多尿症状。最近,他发展为肾病综合征,伴有小腿水肿和蛋白尿(24小时尿中白蛋白14克)。IgE浓度(250国际单位/毫升)和IgA浓度(745毫克/分升)升高,嗜酸性粒细胞增多达14%。肾穿刺活检显示为轻度增殖性肾小球肾炎。切除部分结节性肿瘤后,组织学检查显示为木村病的典型表现(皮肤嗜酸性滤泡性淋巴结炎;伴有嗜酸性粒细胞增多的皮下血管淋巴样增生)。口服泼尼松龙每日20毫克治疗期间,临床和生化指标在两周内恢复正常。但八周后,剂量减至每日10毫克时,肾病综合征复发,因此剂量不得不再次增加至每日20毫克泼尼松龙。经此治疗,患者目前已无症状六个月。该病例显示了木村病与轻度增殖性肾小球肾炎的不寻常关联。