Rheumatology Department, Faculty of Medicine, Cairo University, Kasr Al-Ainy St., Cairo, 11562, Egypt.
Radiology Department, Faculty of Medicine, Cairo University, Kasr Al-Ainy St., Cairo, 11562, Egypt.
Clin Rheumatol. 2021 Dec;40(12):4993-5008. doi: 10.1007/s10067-021-05912-3. Epub 2021 Sep 17.
Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease.
The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type.
The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image selection and approved by HSSISG board members. We classified these findings according to the clinical course of the patients.
This atlas describes the CTPA images that best define the wide spectrum of pulmonary vasculitis observed in HSS. Pulmonary aneurysms were classified into six radiographic patterns: from true stable PAA with adherent in-situ thrombosis to unstable leaking PAA, BAA and/or PAP with loss of aneurysmal wall definition (most prone to rupture), also CTPA images demonstrating right ventricular strain and intracardiac thrombosis.
The HSSISG reference atlas is a guide for physicians regarding the CTPA radiological findings, essential for early diagnosis and management of HSS-related pulmonary vasculitis. Key Points • The Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by extensive vascular thrombosis and pulmonary artery aneurysms (PAAs) that can lead to significant morbidity and mortality. • All fatalities reported in HSS were related to unpredictable massive hemoptysis; therefore, it is critical to recognize pulmonary complications at an early stage of the disease. • The HSS International Study Group reference atlas classifies pulmonary vasculitis in HSS at 6 different stages of the disease process and defines the different radiological patterns of pulmonary vasculitis notably pulmonary artery aneurysms, as detected by computed tomography pulmonary angiography (CTPA). • The main aim of the classification is to make a guide for physicians about this rare syndrome. Such a scheme has never been reached before since the first description of the syndrome by Hughes and Stovin since 1959. This classification will form the basis for future recommendations regarding diagnosis and treatment of this syndrome.
休斯-斯托文综合征（HSS）是一种以广泛的静脉/动脉血栓形成和肺动脉瘤（PAA）为特征的系统性血管炎，与严重的发病率和死亡率相关。HSS 报告的所有死亡均源于不可预测的致命性咯血窒息。因此，有必要在疾病的早期阶段识别肺部并发症。
本研究旨在通过计算机断层肺动脉造影（CTPA）创建一个描绘 HSS 特征性表现的图像参考图谱。根据每种不同病变类型相关的严重程度和并发症风险，制定 PAA 分类，为医生提供指导。
HSS 国际研究组（HSSISG）的成员在一个放射科站点收集了 42 例高质量的 CTPA 图像，并对原始图像进行了重建。对这些详细的 CTPA 研究进行了回顾，以选择最终的图像并获得 HSSISG 董事会成员的批准。我们根据患者的临床病程对这些发现进行了分类。
该图谱描述了可以最佳定义 HSS 中观察到的广泛肺部血管炎的 CTPA 图像。肺动脉瘤分为六种影像学类型：从有原位血栓附着的真性稳定 PAA 到不稳定的渗漏 PAA、BAA 和/或 PAP，其动脉瘤壁定义丧失（最容易破裂），也有 CTPA 图像显示右心室应变和心内血栓。
HSSISG 参考图谱是医生在 CTPA 影像学发现方面的指南，对于早期诊断和管理 HSS 相关的肺部血管炎至关重要。关键点：
休斯-斯托文综合征（HSS）是一种以广泛血管血栓形成和肺动脉瘤（PAA）为特征的系统性血管炎，可导致显著的发病率和死亡率。
HSS 报告的所有死亡均与不可预测的大量咯血有关；因此，在疾病早期识别肺部并发症至关重要。
HSS 国际研究组参考图谱根据疾病过程的 6 个不同阶段对 HSS 中的肺部血管炎进行分类，并定义了通过计算机断层肺动脉造影（CTPA）检测到的不同肺部血管炎的影像学模式，尤其是肺动脉瘤。
分类的主要目的是为医生提供关于这种罕见综合征的指南。自 1959 年 Hughes 和 Stovin 首次描述该综合征以来，这是第一次对该综合征进行如此分类。该分类将为未来关于该综合征的诊断和治疗建议奠定基础。
