Abstract:To describe the pattern of pulmonary artery vasculitis and the characteristic computed tomographic pulmonary angiography (CTPA) signs in patients with Hughes-Stovin syndrome (HSS). In a retrospective study, the medical records of eight HSS patients (six men), seen between February 2008 and January 2018, were reviewed regarding history, disease characteristics, laboratory investigations, imaging, and treatments. The mean (SD) age was 37.375 ± 8.65 years (range 30-55) and mean (SD) follow-up 30 ± 41.60 months (r… Show more
“…Despite the classic clinical and characteristic CTPA findings of HSS, delayed diagnosis is common and misdiagnosis for isolated PTE may occur [ 10 – 41 ]. To facilitate earlier diagnosis and reduce the potential morbidity associated with misdiagnosis, the HSSISG assembled CTPA images into this reference atlas that we believe demonstrate all potential pulmonary complications associated with HSS.…”
Section: Discussionmentioning
confidence: 99%
“…These classifications were developed according to lesion severity and the potential morbidity associated with each individual lesion. PAAs are considered to be one of the most consistent signs of pulmonary vasculitis by CTPA imaging in both HSS [ 10 – 41 ] and Behçet’s disease (BD) [ 42 ]. In our opinion, the earliest CTPA sign of HSS-related pulmonary vasculitis is arterial wall enhancement of the mural wall of the PAA (Fig.…”
Section: Discussionmentioning
confidence: 99%
“…In addition to true PAA in HSS, some older reports noted pulmonary artery pseudo-aneurysms (PAPs) with destruction of a large portion of the false aneurysm’s wall and direct communication from the lumen to the adjacent bronchus, which eventually resulted in fatal hemoptysis [ 7 – 9 ]. These classical autopsy findings can currently be evaluated at an earlier stage using computed tomography pulmonary angiography (CTPA) imaging, which also may provide opportunities for early, targeted management of these pulmonary lesions [ 10 – 41 ].…”
Introduction
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.
Objectives
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.
Methods
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.
Results
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.
Conclusion
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.
“…Despite the classic clinical and characteristic CTPA findings of HSS, delayed diagnosis is common and misdiagnosis for isolated PTE may occur [ 10 – 41 ]. To facilitate earlier diagnosis and reduce the potential morbidity associated with misdiagnosis, the HSSISG assembled CTPA images into this reference atlas that we believe demonstrate all potential pulmonary complications associated with HSS.…”
Section: Discussionmentioning
confidence: 99%
“…These classifications were developed according to lesion severity and the potential morbidity associated with each individual lesion. PAAs are considered to be one of the most consistent signs of pulmonary vasculitis by CTPA imaging in both HSS [ 10 – 41 ] and Behçet’s disease (BD) [ 42 ]. In our opinion, the earliest CTPA sign of HSS-related pulmonary vasculitis is arterial wall enhancement of the mural wall of the PAA (Fig.…”
Section: Discussionmentioning
confidence: 99%
“…In addition to true PAA in HSS, some older reports noted pulmonary artery pseudo-aneurysms (PAPs) with destruction of a large portion of the false aneurysm’s wall and direct communication from the lumen to the adjacent bronchus, which eventually resulted in fatal hemoptysis [ 7 – 9 ]. These classical autopsy findings can currently be evaluated at an earlier stage using computed tomography pulmonary angiography (CTPA) imaging, which also may provide opportunities for early, targeted management of these pulmonary lesions [ 10 – 41 ].…”
Introduction
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.
Objectives
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.
Methods
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.
Results
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.
Conclusion
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.
“…PTE, on the other hand, are serpiginous and extremely mobile, traveling through the bloodstream from the deep venous system to lodge in the pulmonary artery branches. Taken together, in situ thrombosis should be the official radiological term to use in order to define the adherent intraluminal thrombosis seen in HSSrelated pulmonary vasculitis [5,7,8].…”
“…Cases of Hughes‐Stovin syndrome have been found predominantly in male patients with Behçet’s syndrome. With routine use of CT pulmonary angiography, the need for catheter pulmonary angiography is reduced (2,3). Treatment approaches involving immunomodulator therapy have been shown to reverse many of the changes occurring in Hughes‐Stovin syndrome.…”
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
