狼疮性呼吸系统危象表现

Clinical manifestation of lupus respiratory systemic crisis in children

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种多发于女性、累及多脏器的自身免疫性结缔组织病,SLE合并呼吸系统病变在儿童起病急、病情重,有潜在的致命性。因此,要认识其复杂性和严重性,特别要注意狼疮性呼吸系统危象表现的发生。狼疮性呼吸系统危象主要指的是:①急性狼疮性肺炎,②狼疮性肺出血。两者发病率虽较低,但病死率高,如能尽早结合临床症状和体征、胸部X线、肺功能与病理改变及时明确诊断,同时给予尽快尽早使用大剂量皮质激素及免疫抑制剂的治疗,可明显提高近期生存率,改善预后。

Systemic lupus erythematosus （SLE） is an autoimmune connective tissue disease that primarily affects young women and involves in multiple organs. There usually is acute onset and severe states even with potential fatality in children with SLE associated with respiratory systemic disease. It is necessary to realize its complexity and severity, especially to recognize the occurrence of the lupus respiratory systemic crisis. The lupus respiratory systemic crisis primarily includes： （1）lacute lupus pneumonia, and （2） lupus pulmonary hemorrhage. Although the morbidity is lower but the mor- tality is high. If the diagnosis could be confirmed timely based on clinical symptoms, chest X-ray, pulmonary function and pathological changes; and large dose cortical hormone and immunosuppressant can be given as soon as possible following diagnosis, it will be feasible to increase the short-term survival rate and to improve prognosis obviously in children with lupus respiratory systemic crisis.