Matousovic K, Martínek V
Interní klinika 2. lékarské fakulty UK a FN Motol, Praha.
Vnitr Lek. 2003 Nov;49(11):869-73.
IgA nephropathy is the most frequent primary glomerulonephritis worldwide. At its onset, the most common laboratory sign is isolated haematuria often accompanied with mild proteinuria (up to 1.5 g/24 h). The disease displays a progressive course with end-stage renal disease occurring in up to half of patients 20 years after onset. Diagnosis is established by immunofluorescent microscopy of a renal biopsy specimen. Discoveries in the past decade on the pathogenesis of IgA nephropathy together with complex evaluation of its clinical presentation enable to establish diagnosis with a satisfactory degree of probability even without biopsy. IgA nephropathy patients display increased or borderline serum IgA levels; increased serum levels of IgA fraction with degalactosylated O-linked side sugar chains; increased serum levels of anti-N-acetylgalactosamine antibodies; increased levels of circulating immune complexes composed of IgA1 complexed with IgG or IgA1; increased serum levels of circulating complexes composed of IgA and fibronectin; and frequent occurrence of the rheumatoid IgA factor. Clinical use of these still generally unavailable methods would reduce the renal biopsy indication in patients with isolated or predominant haematuria.
IgA肾病是全球最常见的原发性肾小球肾炎。发病时,最常见的实验室指标是单纯血尿,常伴有轻度蛋白尿(高达1.5g/24小时)。该疾病呈进行性发展,发病20年后高达半数患者会发展为终末期肾病。通过肾活检标本的免疫荧光显微镜检查来确诊。过去十年对IgA肾病发病机制的研究以及对其临床表现的综合评估,使得即使不进行活检也能以较高的概率确诊。IgA肾病患者血清IgA水平升高或处于临界值;具有去半乳糖基化O-连接侧糖链的IgA组分血清水平升高;抗N-乙酰半乳糖胺抗体血清水平升高;由IgA1与IgG或IgA1复合而成的循环免疫复合物水平升高;由IgA和纤连蛋白组成的循环复合物血清水平升高;类风湿IgA因子频繁出现。临床应用这些目前普遍尚未可用的方法将减少孤立性或主要为血尿患者的肾活检指征。