Saxena R, Bygren P, Arvastson B, Wieslander J
Department of Nephrology, University Hospital of Lund, Sweden.
J Intern Med. 1995 Aug;238(2):143-52. doi: 10.1111/j.1365-2796.1995.tb00912.x.
Pulmonary renal syndrome (lung haemorrhage and glomerulonephritis) is a fulminant condition that warrants a rapid diagnosis and treatment to prevent mortality and preserve renal functions. However, the patients frequently present with non-specific pulmonary symptoms in the early phase of the syndrome and the diagnosis is often missed. Recently, several autoantibodies have been described in association with various forms of glomerulonephritis. We evaluated the association as well as the diagnostic and the prognostic significance of these antibodies in pulmonary renal syndrome.
Retrospective clinical study.
University Hospital.
Forty consecutive patients with biopsy verified glomerulonephritis and overt haemoptysis or pulmonary infiltrates compatible with lung haemorrhage.
Analysis of proteinase 3 antineutrophil cytoplasm antibodies (PR3-ANCA), myeloperoxidase (MPO)-ANCA, antiglomerular basement membrane (GBM) and anti-entactin antibodies.
Thirty-six (90%) patients possessed one or more autoantibodies. Twenty-seven (70%) patients had ANCA (PR3-ANCA, MPO-ANCA or both). The remaining positive patients (n = 9) had anti-GBM antibodies. Only two patients had anti-entactin antibodies, suggesting a poor association of these antibodies with PRS. The majority of patients with anti-GBM antibodies had a very poor clinical outcome (five irreversible renal failure; three deaths). On the other hand, despite no significant difference in clinical features or renal morphology from patients with anti-GBM antibodies, 19 patients (70%) with ANCA recovered completely following treatment.
Our study demonstrated that the presence of autoantibodies is a predominant feature of PRS and that the type of immunologic injury is of paramount importance in determining the course of illness in this syndrome. Analysis of the aforementioned antibodies can help in an early differential diagnosis and thus, in better management of PRS.
肺肾综合征（肺出血和肾小球肾炎）是一种严重疾病，需要迅速诊断和治疗以防止死亡并保留肾功能。然而，患者在该综合征早期常表现出非特异性肺部症状，诊断往往被遗漏。最近，已发现几种自身抗体与各种形式的肾小球肾炎有关。我们评估了这些抗体在肺肾综合征中的关联性以及诊断和预后意义。
回顾性临床研究。
大学医院。
40例经活检证实为肾小球肾炎且有明显咯血或与肺出血相符的肺部浸润的连续患者。
分析蛋白酶3抗中性粒细胞胞浆抗体（PR3-ANCA）、髓过氧化物酶（MPO）-ANCA、抗肾小球基底膜（GBM）抗体和抗巢蛋白抗体。
36例（90%）患者拥有一种或多种自身抗体。27例（70%）患者有ANCA（PR3-ANCA、MPO-ANCA或两者兼有）。其余阳性患者（n = 9）有抗GBM抗体。只有2例患者有抗巢蛋白抗体，表明这些抗体与肺肾综合征的关联性较差。大多数抗GBM抗体阳性患者的临床结局非常差（5例不可逆肾衰竭；3例死亡）。另一方面，尽管与抗GBM抗体阳性患者在临床特征或肾脏形态上无显著差异，但19例（70%）ANCA阳性患者治疗后完全康复。
我们的研究表明，自身抗体的存在是肺肾综合征的主要特征，免疫损伤类型在决定该综合征的病程中至关重要。对上述抗体的分析有助于早期鉴别诊断，从而更好地管理肺肾综合征。
