目的:描述灾难性抗磷脂综合征(CAPS)患者的肺部受累,关注它与肺外受累的关系,实验室,放射学,和病理结果。
方法:本回顾性横断面研究包括“CAPS登记处”中的所有患者。对所有病例进行了审查,选择患有肺血栓栓塞症(PE)和/或弥漫性肺泡出血(DAH)的患者。肺部和肺外临床表现的数据,放射学模式,实验室发现,相关的自身免疫性疾病,治疗,并对结果进行了分析。使用频率分布和集中趋势度量来描述队列。组间关于定性变量的比较采用卡方检验或Fisher精确检验,而独立变量的T检验用于比较连续变量的组。使用IBM-SPSSv.22进行数据分析。
结果:PE在129(48.6%)发作中报告,DAH在75(28.3%)发作中,在7次(2.6%)发作中重叠(DAH加PE)。在35(4.9%)CAPS发作中进行了支气管肺泡灌洗(BAL),在84次(10.5%)发作(包括尸检)中获得了肺病理学样本。在DAH与血栓性微血管病(TMA)的实验室特征之间观察到显着关系。在三重抗磷脂抗体阳性与病理性TMA(26.5%)以及低补体血症和DAH(24%)之间也发现了有意义的关系。
结论:肺部受累可能包括TMA和非血栓性炎症,可以分为三种模式:PE,DAH伴全身性TMA伴低补体血症或DAH不伴/不伴低补体血症的全身性TMA。
To describe the pulmonary involvement in patients with catastrophic antiphospholipid syndrome (CAPS), focusing on its relationship with extrapulmonary involvement, laboratory, radiological, and pathological findings.
This retrospective cross-sectional study includes all patients grouped in the \"CAPS Registry\". All cases were reviewed, and those with pulmonary thromboembolism (PE) and/or diffuse alveolar hemorrhage (DAH) were selected. Data on pulmonary and extrapulmonary clinical presentation, radiologic patterns, laboratory findings, associated autoimmune diseases, treatments, and outcomes were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test, while T-test for independent variables was used to compare groups regarding continuous variables. IBM-SPSS v.22 was used for data analysis.
PE was reported in 129 (48.6 %) episodes, DAH in 75 (28.3 %) episodes, and overlap (DAH plus PE) in 7 (2.6 %) episodes. Bronchoalveolar lavage (BAL) was performed in 35 (4.9 %) CAPS episodes, and lung pathology samples were obtained in 84 (10.5 %) episodes (including autopsies). A significant relationship was observed between DAH and laboratory features of thrombotic microangiopathy (TMA). A meaningful relationship was also found between triple antiphospholipid antibody positivity and pathological TMA (26.5 %) as well as hypocomplementemia and DAH (24 %).
Pulmonary involvement may include both TMA and non-thrombotic inflammation, which can be differentiated into three patterns: PE, DAH with systemic TMA with hypocomplementemia or DAH without systemic TMA with/without hypocomplementemia.