Acquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD). AVWS is secondary to other diseases, and systemic lupus erythematosus (SLE) is a relatively rare cause.

We report a case of AVWS as onset clinical presentation of SLE manifested as epistaxis and pulmonary hemorrhage. A 13-year-old male child presented to the hospital with a six-month history of recurrent epistaxis and a one-month history of anemia. Routine blood tests demonstrated severe normocytic anemia and normal platelet count. Von Willebrand test revealed a significantly lower level. High-resolution chest computed tomography (CT) showed patchy ground glass opacities consistent with hemorrhagic changes. After ruling out the family history, the patient was diagnosed with AVWS. Additional tests confirmed positive antinuclear and anti-Sm antibodies. The underlying SLE was diagnosed and treated with methylprednisolone with disease recovery.

We recommend screening for bleeding disorders in patients with recurrent epistaxis. AVWS should be considered when laboratory findings suggest hereditary von Willebrand disease without a personal or familial history of bleeding. In addition, the underlying disease should be explored.

获得性血管性血友病综合征 (AVWS) 是一种不太常见的出血性疾病，主要表现为轻度至中度皮肤黏膜出血，实验室检查与遗传性血管性血友病 (VWD) 相似。AVWS继发于其他疾病，系统性红斑狼疮（SLE）是一种相对罕见的病因。

我们报告了一例 AVWS 作为 SLE 的临床表现，表现为鼻出血和肺出血。一名 13 岁男孩因反复鼻出血 6 个月和贫血 1 个月就诊。常规血液检查显示严重的正细胞性贫血和正常的血小板计数。Von Willebrand 测试显示显着较低的水平。高分辨率胸部计算机断层扫描 (CT) 显示与出血性变化一致的斑片状磨玻璃影。排除家族史后，确诊为AVWS。其他测试证实抗核抗体和抗 Sm 抗体呈阳性。潜在的 SLE 被诊断并用甲基强的松龙治疗，疾病恢复。

我们建议对复发性鼻出血患者进行出血性疾病筛查。当实验室检查结果提示遗传性血管性血友病而无个人或家族出血史时，应考虑 AVWS。此外，应探索潜在的疾病。