Song J H, Lee K J, Lee S W, Han J Y, Kim M J
Department of Internal Medicine, Inha University College of Medicine, Inchon, Korea.
Yonsei Med J. 2001 Aug;42(4):425-30. doi: 10.3349/ymj.2001.42.4.425.
Recent reports have indicated that a significant number of immune complex glomerulonephritis (GN) cases are associated with antineutrophilic cytoplasmic antibody (ANCA). However, most of the reported cases were associated with underlying primary glomerular diseases. When primary glomerular diseases were not found, immune deposits tended to be non-specific and the level of ANCA is usually borderline. We report here upon a case of life-threatening pulmonary-renal syndrome manifested simultaneously with immune complex GN and myeloperoxidase (MPO)-ANCA seropositivity. A 29- year-old man was admitted with pulmonary hemorrhage and rapidly progressing renal dysfunction. On admission, ANCA revealed perinuclear staining with a titer of 1:160. The MPO-ANCA level was 59 IU by ELISA. Other serologic markers including ANA, anti-DS-DNA and anti-GBM Ab were negative. Renal biopsy showed cellular crescents in eight of 18 glomeruli. Immunofluorescence staining showed strong granular deposits of C3, C1q, IgG and IgM in the capillary loop and the mesangium. Electron microscopy showed multifocal electron dense deposits scattered in the mesangium, paramesangium, and the subendothelial and subepithelial areas. The patient initially responded to steroid and cyclophosphamide. MPO-ANCA decreased to less than 10 IU. Twenty three days after hospital discharge, the patient was re-admitted urgently with fever, generalized papulonodular skin lesions, and a recurrence of massive pulmonary hemorrhage and renal dysfunction. He died from uncontrolled pulmonary hemorrhage and respiratory insufficiency. P-ANCA titer and MPO-ANCA level at the second admission were 1:320 and 82 U/ml respectively. Interestingly, relapse was shown to be triggered by varicella zoster infection.
近期报告显示，相当数量的免疫复合物肾小球肾炎（GN）病例与抗中性粒细胞胞浆抗体（ANCA）相关。然而，大多数报告病例与潜在的原发性肾小球疾病有关。当未发现原发性肾小球疾病时，免疫沉积物往往是非特异性的，且ANCA水平通常处于临界值。我们在此报告一例危及生命的肺肾综合征病例，该病例同时表现为免疫复合物GN和髓过氧化物酶（MPO）-ANCA血清阳性。一名29岁男性因肺出血和快速进展的肾功能不全入院。入院时，ANCA显示核周染色，滴度为1:160。通过酶联免疫吸附测定法（ELISA）测得MPO-ANCA水平为59国际单位。包括抗核抗体（ANA）、抗双链DNA（anti-DS-DNA）和抗肾小球基底膜抗体（anti-GBM Ab）在内的其他血清学标志物均为阴性。肾活检显示18个肾小球中有8个出现细胞性新月体。免疫荧光染色显示C3、C1q、IgG和IgM在毛细血管袢和系膜区有强烈的颗粒状沉积。电子显微镜显示多灶性电子致密沉积物散在于系膜、系膜旁、内皮下和上皮下区域。患者最初对类固醇和环磷酰胺有反应。MPO-ANCA降至10国际单位以下。出院23天后，患者因发热、全身丘疹结节性皮肤病变、大量肺出血和肾功能不全复发而紧急再次入院。他死于无法控制的肺出血和呼吸功能不全。第二次入院时P-ANCA滴度和MPO-ANCA水平分别为1:320和82 U/ml。有趣的是，复发被证明是由水痘带状疱疹感染引发的。
