巨噬细胞活化综合征(MAS)是一种继发于风湿免疫疾病的噬血细胞性淋巴组织细胞增多症(HLH),是一种罕见的、危及生命的过度炎症性疾病。巨噬细胞活化综合征的典型临床症状包括持续高热、皮疹以及肝和脾的肿大,实验室的检测结果显示患者的血细胞数量减少,伴有高甘油三酯或低纤维蛋白原、高铁蛋白血症和转氨酶水平上升等现象。在全身性幼年特发性关节炎(sJIA)和成人发病斯蒂尔氏病(AOSD)中,巨噬细胞活化综合征的发病率较高,但目前有越来越多的学者对系统性红斑狼疮患者发生巨噬细胞活化综合征进行了报道。巨噬细胞活化综合征病情复杂,进展凶险,若误诊或延误治疗时机,可能造成无法挽回的不良结果,所以早期识别巨噬细胞活化综合征并进行诊治就显得十分必要。本文通过检索Pubmed数据库,对系统性红斑狼疮合并巨噬细胞活化综合征的诊治进展做一综述。Macrophage activation syndrome (MAS), a hemophagocytic lymphohistiocytosis (HLH) secondary to rheumatic immune disease, is a rare, life-threatening hyperinflammatory disease. Typical clinical symptoms of macrophage activation syndrome include persistent high fever, rash, and enlargement of the liver and spleen, and laboratory tests show a decrease in the number of blood cells in the patient, with high triglycerides or low fibrinogen, ferritinemia, and elevated aminotransferase levels. The incidence of macrophage activation syndrome is high in systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), but more and more scholars have reported the occurrence of macrophage activation syndrome in patients with systemic lupus erythematosus. Macrophage activation syndrome is complex and progresses dangerously, and if it is misdiagnosed or delayed, it may cause irreversible adverse results, so it is necessary to identify and treat macrophage activation syndrome early. This article reviews the progress in the diagnosis and treatment of systemic lupus erythematosus complicated with macrophage activation syndrome by searching the Pubmed database.
耶氏肺孢子虫肺炎(Pneumocystis Jirovecii Pneumonia, PJP)是一种机会性感染,其发病率低但死亡率高,既往多发于人类免疫缺陷病毒(Human Immunodeficiency Virus, HIV)患者中。由于免疫抑制治疗的广泛使用,PJP已成为免疫功能低下的非HIV感染患者的新威胁。PJP患者的临床表现、诊断方法、危险因素、治疗及预后在HIV患者及非HIV患者中不完全相同。在非HIV患者中PJP往往进展迅速,容易出现呼吸衰竭导致预后不良。PJP的诊断方法有多种包括肺囊虫镜检、聚合酶链反应(PCR)、宏基因组二代测序(NGS),但上述方法均为有创操作且适用范围受限。血清学检查β-D葡聚糖( beta-D glucan, β-DG)是一种较为简便的无创操作,有较高的灵敏度及阴性预测值,目前越来越多地运用于PJP的辅助诊断。因合并PJP往往导致较差结局,故寻找其危险因素很重要,以便早期预防及治疗。有研究表明PJP好发于特发性炎性肌病(Idiopathic Inflammatory Myopathies, IIM)患者中,故本文对PubMed及知网数据库进行了全面的文献检索,对特发性炎性肌病合并耶氏肺孢子虫肺炎患者的发病机制、临床特征、诊断方法、危险因素、治疗及预防等方面做一个系统阐述。Pneumocystis jirovecii pneumonia (PJP) is an opportunistic infection with low morbidity but high mortality that has been prevalent in patients with human immunodeficiency virus (HIV). Due to the widespread use of immunosuppressive therapy, PJP has emerged as a new threat to immunocompromised non-HIV-infected patients. The clinical presentation, diagnostic approach, risk factors, treatment, and prognosis in patients with PJP are not identical in HIV patients and non-HIV patients. In non-HIV patients, PJP tends to progress rapidly and is prone to respiratory failure, leading to a poor prognosis. There are a variety of methods for the diagnosis of PJP, such as microscopic examination of pneumocystis, polymerase chain reaction (PCR), and metagenomic next-generation sequencing (NGS), but these methods are invasive and have limited applicability. Serological beta-D glucan (β-DG) is a relatively simple and non-invasive operation with high sensitivity and negative predictive value, which is increasingly used as an auxiliary diagnosis of PJP. Because the combination of PJP tends to lead to worse outcomes, it is important to look for risk factors for early prevention and treatment. Studies have shown that PJP is more common in idiopathic inflammatory myopathy (IIM), so this paper conducted a comprehensive literature search of PubMed and CNKI databases to systematically elaborate on the pathogenesis, clinical features, dia
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