特刊文章-临床肾脏学和治疗学杂志(2021)第0卷，第0期

抗肾小球基底膜病和good牧草病

Goodpasture病也被称为抗gbm病，与IV型胶原蛋白α 3链的高水平循环IgG自身抗体相关。在高加索人群中，好牧草病的发生更为普遍，每年达到百万分之一。累及肺部的抗gbm疾病在男性中更为普遍，约80%通常发生在第二个十年，而孤立的抗gbm肾炎也可发生在老年人中，但没有男性优势。许多有肺出血急性症状的患者可能同时伴有肾脏疾病或单独有肺出血症状，包括咳嗽、呼吸困难、咯血和缺铁性贫血。肾小球肾炎累及肾脏可表现为深色和红色尿液，但进展到少尿的速度很快，因此可跳过这一步。肾小球肾炎发生在三分之一或一半的患者没有肺出血。一旦发生严重的肾功能损害，病情通常会迅速恶化。尿检常见血尿、中度蛋白尿、显微镜下红细胞畸形和红细胞铸型(甚至与肺部疾病明显相关)。肾活检是重要的，因为它提供诊断和预后信息，典型的组织学特征是弥漫性增殖性肾小球肾炎伴不同程度的坏死，新月形形成，肾小球硬化和肾小管丢失。免疫球蛋白沿肾小球基底膜呈线性沉积是典型的。 IgG and anti-GBM antibodies that are circulating are almost always present. The titers of anti-GBM antibodies correlate with the intensity of nephritis. Shift in titer also represents treatment and relapses. Anti-GBM antibodies often associated with ANCA , especially ANCA myeloperoxidase, in such double positive patients may have a clinical course and response to treatment more usually vasculitis than Goodpasture disease. Recovery of renal function is likely even patient is dialysis dependent. For most patients, anti-GBM disease treatment should be begun as soon as possible. However, the chances of recovery and independent kidney function in patients with some clinical and pathological conditions are poor. Kidney function recovery is only 5 percent in patients who have a high percentage of crescent on kidney biopsy (85- 100 percent). Oliguria and/or advanced kidney failure that needs dialysis to begin, should consider before initiation of immunosuppressive therapy due to low chance of kidney recovery and the ability of the patients to withstand intense immunosuppression but treatment is appropriate for those patients who have pulmonary haemorrhage. The purpose of the treatment is to suppress kidney inflammation, remove circulating pathogenic autoantibodies and suppressing autoantibody formation. This treatment can prevent ongoing kidney damage but is unable to reverse the chronic kidney damage already developed. In most patients treated with plasma exchange combined with immunosuppression, antibodies are eliminated within eight weeks . Plasma exchange removes anti-GBM antibodies from the bloodstream steadily and slowly (within several weeks) and usually must be achieved for two to three weeks before anti-GBM antibodies are eliminated completely . Cyclophosphamide for 3 months and eventually tapered corticosteroids fully removed within 6 months tend to be enough based on available clinical data. After 3 months of cyclophosphamide, continuation of treatment with either Azathioprine or Mycophenolate is indicated in patients with persistent anti-GBM antibodies. Anti-GBM disease relapses are rare. After renal transplant, recurrence of anti-GBM disease maybe 50 percent for those who have detectable anti-GBM antibodies at the time of renal transplant [6]. It is not recommended to treat patients who do not have detectable anti-GBM antibodies for more than six months.

作者:Syed Shabbar Musavi

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