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AB038. Isolated pauci-immune pulmonary capillaritis: rare case of diffuse alveolar hemorrhage

机译:AB038。单纯性小免疫性肺毛细血管炎:弥漫性肺泡出血的罕见病例

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摘要

As diffuse alveolar hemorrhage is defined the clinical pathological syndrome characterized by hemoptysis, diffuse alveolar infiltrates, acute respiratory failure and anemia. It is a life-threatening condition and a medical emergency. Causes are multiple and variable. A 75-year-old male, ex-smoker, with known coronary artery disease and paroxysmal atrial fibrillation treated with aspirin, presents due to low grade fever and bloody sputum. Hemodynamically stable, without respiratory failure, but with multiple alveolar infiltrates on chest imaging and anemia. Initially treated as lower respiratory tract infection with intravenous antibiotics while anticoagulant treatment was interrupted. Full laboratory testing for anemia, collagen vascular diseases and specific and nonspecific infections was performed. Anemia was normochromic, normocytic, without findings of hemolysis. Diffuse bilateral alveolar infiltrates were revealed on thorax computer tomography. Diagnostic bronchoscopy was performed with negative results for either infection or malignancy, while bronchoalveolar lavage analysis revealed siderophages 81% of alveolar macrophages. The patient underwent full pulmonary function testing, which revealed obstructive respiratory disease with a low diffusion capacity. Due to continuing life threatening anemia, despite blood transfusions, in combination with the results of the laboratory tests, the patient was started on high dose intravenous corticosteroids, followed by intravenous cyclophosphamide every 28 days for five cycles. Rapid improvement was achieved with elimination of chest CT infiltrates and normalization of blood tests and pulmonary function tests. At present peros treatment with azathioprine and low dose corticosteroids is given. Diffuse alveolar hemorrhage is a clinical pathological syndrome caused by different pathophysiological mechanisms, including capillary failure stress, diffuse alveolar damage and capillaritis. The most common cause is capillaritis associated with systemic autoimmune diseases, mostly ANCA-related vasculitides. The presence of diffuse alveolar hemorrhage, with pulmonary capillaritis without clinical or serological findings of a systemic autoimmune disease is known as isolated pauci-immune pulmonary capillaritis. The treatment is based on corticosteroids and cyclophosphamide.
机译:弥漫性肺泡出血定义为以咯血,弥漫性肺泡浸润,急性呼吸衰竭和贫血为特征的临床病理综合征。这是威胁生命的状况和紧急医疗情况。原因是多种多样的。一名75岁的男性,前吸烟者,患有已知的冠状动脉疾病,并接受阿司匹林治疗阵发性房颤,原因是低烧和痰中带血。血流动力学稳定,无呼吸衰竭,但对胸部成像和贫血有多个肺泡浸润。最初以静脉内抗生素治疗为下呼吸道感染,但抗凝治疗中断。进行了贫血,胶原蛋白血管疾病以及特异性和非特异性感染的完整实验室测试。贫血为常色,正常血红细胞,无溶血现象。胸部计算机断层扫描显示双侧弥漫性肺泡浸润。诊断性支气管镜检查对感染或恶性肿瘤均呈阴性结果,而支气管肺泡灌洗分析显示81%的肺泡巨噬细胞为噬菌体。患者接受了全面的肺功能测试,发现阻塞性呼吸道疾病扩散能力低。尽管输血,由于持续的危及生命的贫血,结合实验室测试的结果,患者开始使用大剂量静脉注射皮质类固醇激素,然后每28天静脉注射环磷酰胺五个疗程。通过消除胸部CT浸润以及血液检查和肺功能检查正常化,可以实现快速改善。目前,给予硫唑嘌呤和低剂量皮质类固醇的佩罗斯治疗。弥漫性肺泡出血是由不同病理生理机制引起的临床病理综合征,包括毛细血管衰竭应激,弥漫性肺泡损伤和毛细血管炎。最常见的原因是与全身性自身免疫性疾病(主要是与ANCA相关的血管炎)相关的毛细血管炎。没有全身性自身免疫性疾病的临床或血清学发现的弥漫性肺泡出血,肺毛细血管炎的存在被称为孤立的小剂量免疫性肺毛细血管炎。治疗基于皮质类固醇和环磷酰胺。

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