Knotková V, Krystůfková O
II. interní klinika 1. LF UK, Praha.
Vnitr Lek. 1993 Jul;39(7):706-13.
The authors describe their experience with the treatment of nine patients where "essential" mixed cryoglobulinaemia was diagnosed. In addition to extrarenal symptoms all suffered from proliferative glomerulonephritis, most frequently mesangiocapillary. The latter was manifested in most instances during the first examination by nephrotic syndrome and reduced glomerular filtration. Two patients had only asymptomatic proteinuria with erythrocyturia. The majority suffered from arterial hypertension. The patients were treated with prednisone and cyclophosphamide, using initial doses after which the extrarenal symptoms receded promptly. Regression of proteinuria and normalization of glomerular filtration developed more slowly. Only in one patient the glomerulonephritis had a progressive course and terminated by chronic renal failure. One patient died from septicaemia and one from cardiac failure. The authors assume that early diagnosis and effective combined immunosuppression can ensure a favourable course of this rare disease.
作者描述了他们对9例被诊断为“原发性”混合性冷球蛋白血症患者的治疗经验。除肾外症状外,所有患者均患有增殖性肾小球肾炎,最常见的是系膜毛细血管性肾小球肾炎。后者在大多数情况下在首次检查时表现为肾病综合征和肾小球滤过率降低。2例患者仅有无症状蛋白尿伴红细胞尿。大多数患者患有动脉高血压。患者接受泼尼松和环磷酰胺治疗,使用初始剂量后肾外症状迅速消退。蛋白尿的消退和肾小球滤过的正常化发展较慢。只有1例患者的肾小球肾炎呈进行性病程并以慢性肾衰竭告终。1例患者死于败血症,1例死于心力衰竭。作者认为早期诊断和有效的联合免疫抑制可确保这种罕见疾病有良好的病程。