Clinique de Médecine Interne, Hôpital Michallon, CHU de Grenoble, Grenoble, France.
Ren Fail. 2011;33(10):1032-6. doi: 10.3109/0886022X.2011.610547. Epub 2011 Aug 25.
A 54-year-old male patient was admitted for acute respiratory distress with fever. He was suffering from chronic sinusitis/rhinitis and had persistent otitis for the past 2 months before admission despite several antibiotics courses. He developed a complex pulmonary involvement (embolism and diffuse alveolar hemorrhage) with acute glomerular disease (proteinuria and hematuria but initially no renal failure). Clinical suspicion of Wegener's granulomatosis was confirmed by the positive high titer of antineutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity) and despite a negative nasal biopsy. Treatment including cyclophosphamide and methylprednisolone intravenous pulses permitted pulmonary recovery over 4 weeks contrasting with the development of rapidly progressive glomerulonephritis and polyneuropathy of lower limbs. Renal biopsy showed pauci-immune crescentic and necrotizing glomerulonephritis. However, despite additional plasma exchanges, acute kidney injury worsened and the patient ended up in dialysis. Such a dissociated evolution was unexpected in this case since pulmonary and renal involvements reflected the same pathological process (small vessels vasculitis/capillaritis) and the same pathogenic mechanism (antiproteinase 3 autoantibodies).
一位 54 岁的男性患者因发热伴急性呼吸窘迫入院。他患有慢性鼻窦炎/鼻炎，在入院前的 2 个月里持续患有中耳炎，尽管已经接受了几次抗生素治疗。他出现了复杂的肺部受累（栓塞和弥漫性肺泡出血），同时伴有急性肾小球疾病（蛋白尿和血尿，但最初没有肾功能衰竭）。尽管鼻活检为阴性，但抗中性粒细胞胞质抗体（c-ANCA 具有抗蛋白酶 3 特异性）的高滴度阳性证实了韦格纳肉芽肿的临床怀疑。包括环磷酰胺和甲基强的松龙静脉冲击在内的治疗在 4 周内使肺部恢复，但随后迅速发展为进行性肾小球肾炎和下肢多发性神经病。肾活检显示寡免疫性新月体和坏死性肾小球肾炎。然而，尽管进行了额外的血浆置换，急性肾损伤仍恶化，患者最终进入透析。在这种情况下，这种分离的演变是出乎意料的，因为肺部和肾脏受累反映了相同的病理过程（小血管血管炎/毛细血管炎）和相同的发病机制（抗蛋白酶 3 自身抗体）。
