Malavieille F, Page M, Ber C-E, Christin F, Bonnet A, Rimmele T
Département d'anesthésie et de réanimation, hôpital Édouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69437 Lyon cedex 03, France.
Département d'anesthésie et de réanimation, hôpital Édouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69437 Lyon cedex 03, France.
Rev Mal Respir. 2014 Sep;31(7):636-40. doi: 10.1016/j.rmr.2013.12.005. Epub 2014 Mar 14.
We report a case of acute pulmonary renal syndrome mimicking septic shock, which led to the diagnosis of granulomatosis with polyangiitis.
A 70-year-old man was hospitalized because of acute kidney injury and acute respiratory distress syndrome with diffuse alveolar hemorrhage associated with a serum procalcitonin level of 18 μg/L. Initially, septic shock was suspected and antibiotic therapy was started. The absence of microbiological isolates and the patient's rapid clinical deterioration prompted laboratory testing for autoimmune disease, which confirmed the diagnosis of granulomatosis with polyangiitis. Immunosuppressive therapy was promptly initiated with corticosteroids, cyclophosphamide and several plasma exchanges, which resulted in a rapid clinical improvement and ICU discharge.
Granulomatosis with polyangiitis is a systemic necrotizing vasculitis with antineutrophil cytoplasmic antibodies, which can present with acute pulmonary renal syndrome, combining acute respiratory distress syndrome and acute kidney injury. This misleading presentation must prompt an autoimmune disease testing in order to yield an early diagnosis of a vasculitis, allowing for timely initiation of immunosuppressive treatment. Serum procalcitonin levels can be markedly elevated and this must not override the possibility of a vasculitis where the patient shows a compatible symptomatology.
我们报告一例疑似脓毒症休克的急性肺肾综合征病例，最终诊断为肉芽肿性多血管炎。
一名70岁男性因急性肾损伤、急性呼吸窘迫综合征伴弥漫性肺泡出血入院，血清降钙素原水平为18μg/L。起初，怀疑为脓毒症休克并开始抗生素治疗。由于未分离出微生物且患者临床病情迅速恶化，遂进行自身免疫性疾病实验室检查，确诊为肉芽肿性多血管炎。随即迅速开始使用糖皮质激素、环磷酰胺进行免疫抑制治疗，并进行了多次血浆置换，患者临床症状迅速改善并从重症监护病房出院。
肉芽肿性多血管炎是一种伴有抗中性粒细胞胞浆抗体的系统性坏死性血管炎，可表现为急性肺肾综合征，合并急性呼吸窘迫综合征和急性肾损伤。这种具有误导性的表现必须促使进行自身免疫性疾病检测，以便早期诊断血管炎，从而及时开始免疫抑制治疗。血清降钙素原水平可能会显著升高，但这不能排除患者出现符合血管炎症状时患血管炎的可能性。
