scholarly journals Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide—a report by the HSS International Study Group

2021 ◽  
Author(s):  
Yasser Emad ◽  
Yasser Ragab ◽  
Cal Robinson ◽  
Sonia Pankl ◽  
Pablo Young ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Artery ◽  
Systemic Vasculitis ◽  
International Study ◽  
Pulmonary Angiography ◽  
Study Group ◽  
Computed Tomography Pulmonary Angiography ◽  
International Study Group ◽  
Pulmonary Vasculitis ◽  
Pulmonary Artery Aneurysms

Abstract 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.

scholarly journals Pattern of pulmonary vasculitis and major vascular involvement in Hughes-Stovin syndrome (HSS): brief report of eight cases

2019 ◽  
Vol 39 (4) ◽  
pp. 1223-1228
Author(s):  
Yasser Emad ◽  
Yasser Ragab ◽  
Ossama Ibrahim ◽  
Ahmed Saad ◽  
Johannes J. Rasker
Keyword(s):  
Pulmonary Artery ◽  
Early Treatment ◽  
Systemic Vasculitis ◽  
Pulmonary Angiography ◽  
Favorable Outcome ◽  
Coagulation Profile ◽  
History Of ◽  
Pulmonary Artery Aneurysms ◽  
Laboratory Investigations

AbstractTo 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 (range 9–132). In all patients, routine laboratory investigations and complete coagulation profile were done. In all, CTPA studies were performed as well as and Doppler ultrasound for suspected deep vein thrombosis (DVT). Four patients had a history of thrombophlebitis, and DVT was observed in all, in two cases bilateral. Arterial thromboses involving popliteal, tibial, common iliac, and femoral arteries were observed in one patient. All patients had mild to moderate hemoptysis, and one had massive hemoptysis. None of the patients had a history of recurrent mouth and/or genital ulcers, uveitis, or arthritis. In all patients, CTPA identified bilateral pulmonary artery aneurysms (PAAs) with adherent in situ thrombosis and mural enhancement in all patients. Lobar PA branches were involved in all patients, segmental in six and main PA in five patients. Proper immunomodulators were initiated early, with favorable outcome; none was treated with TNF-α antagonists. HSS is a systemic vasculitis that may affect virtually all major veins and arteries in patients with normal coagulation profile. PAAs, adherent in situ thrombosis, and mural wall enhancement are characteristic CTPA signs. Early treatment with immunomodulators is essential.Key Points• Hughes Stevin syndrome (HSS) is a systemic vasculitis that may affect virtually all major veins and arteries in patients. It has a normal coagulation profile.• Computed tomography (CT) pulmonary angiography is considered to be the most important diagnostic tool to assess the degree and the extent of the characteristic pulmonary artery aneurysms, and in situ thrombosis, and mural wall enhancement.• It is likely that HSS syndrome is often not recognized and misdiagnosed as deep venous thrombosis (DVT) with pulmonary thromboembolism.• Early treatment with combined immunomodulators is essential to ensure favorable outcome.

scholarly journals The filling defect of pulmonary artery, an imaging finding what we should know

2020 ◽  
Vol 10 (1) ◽  
pp. 204589402091068
Author(s):  
Min Liu ◽  
Xin Cao Tao ◽  
Zhenguo Zhai ◽  
Zhanhong Ma ◽  
Li Zhu ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Magnetic Resonance ◽  
Pulmonary Artery ◽  
Pulmonary Thromboembolism ◽  
Clinical Manifestations ◽  
Pulmonary Angiography ◽  
Resonance Imaging ◽  
Computed Tomography Pulmonary Angiography ◽  
Pulmonary Arterial

The most common cause of pulmonary artery filling defects on computed tomography pulmonary angiography or magnetic resonance imaging is pulmonary thromboembolism, but not infrequently, the presentation of this finding lacks specificity. Given that the morbidity and mortality associated with pulmonary thromboembolism is high, proper diagnosis of the condition is essential. Unusual or more rarely encountered etiologies must be considered when clinical manifestations and imaging findings are inconsistent. With this review, our purpose is to describe possible causes of pulmonary arterial filling defects. We aim to provide clinicians with a comprehensive list of differential diagnoses to facilitate a measured approach to the assessment of pulmonary arterial filling defects on computed tomography pulmonary angiography or magnetic resonance imaging.

scholarly journals Investigation of the Need for Computed Tomography Pulmonary Angiography in the Decision to Discontinue Treatment for Pulmonary Thromboembolism

2021 ◽  
Author(s):  
Eda Çelik ◽  
Ömer Araz ◽  
Buğra Kerget ◽  
Elif Yılmazel Uçar ◽  
Metin Akgün ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Artery ◽  
Sensitivity And Specificity ◽  
Outpatient Clinic ◽  
Pulmonary Thromboembolism ◽  
Pulmonary Angiography ◽  
Treatment Discontinuation ◽  
Computed Tomography Pulmonary Angiography ◽  
Pulmonary Artery Obstruction ◽  
D Dimer

Abstract Purpose: Acute pulmonary thromboembolism (PTE) is an important cause of morbidity and mortality that can reduce quality of life due to long-term complications during and after treatment discontinuation. The aim of this study was to evaluate patients for these complications before discontinuing treatment and determine the necessity of computed tomography pulmonary angiography (CTPA) imaging.Methods: This retrospective study included 116 patients over the age of 18 who received anticoagulant treatment for at least 3 months and presented for treatment discontinuation to the Atatürk University Research Hospital Chest Diseases Outpatient Clinic between January 2015 and September 2019. Results: CTPA performed at treatment discontinuation showed complete thrombus resolution with treatment in 73 patients (62.9%). High pulmonary artery obstruction index (PAOI) at diagnosis was statistically associated with findings of residual or chronic thrombus on CTPA at treatment discontinuation (p=0.001). In the differentiation of patients with residual/chronic thrombus and those with thrombus resolution, D-dimer at a cut-off value of 474 µg/L had 60% sensitivity and 70% specificity. At a cut-off value of 35.5 mmHg, mean pulmonary artery pressure on echocardiography had sensitivity and specificity of 72% and 77%, respectively. At a cut-off of 23.75, PAOI had sensitivity and specificity of 93% and 69%, respectively.Discussion: In addition to physical examination findings, D-dimer and echocardiography were guiding parameters in the evaluation of treatment discontinuation and thrombus resolution in patients presenting to the outpatient clinic for discontinuation of treatment for acute PTE. PAOI at diagnosis may be another important guiding parameter in addition to these examinations.

Simultaneous bilateral contrast injection in computed tomography pulmonary angiography

2012 ◽  
Vol 53 (1) ◽  
pp. 69-75 ◽  
Author(s):  
Stefan Bulla ◽  
Gregor Pache ◽  
Thorsten Bley ◽  
Mathias Langer ◽  
Philipp Blanke
Keyword(s):  
Computed Tomography ◽  
Pulmonary Embolism ◽  
Image Quality ◽  
Pulmonary Artery ◽  
Contrast Media ◽  
Pulmonary Angiography ◽  
Computed Tomography Pulmonary Angiography ◽  
Fixed Amount ◽  
Institutional Review ◽  
Group A

Background Computed tomography pulmonary angiography (CTPA) has evolved as the gold standard for diagnosing pulmonary embolism. However, subsegmental arteries are often not assessed to do insufficient attenuation. Purpose To evaluate the influence of simultaneous bilateral versus unilateral injection of a fixed amount of contrast media on pulmonary artery opacification and image quality in CTPA. Material and Methods In this institutional review board-approved prospective study, 180 patients (91 women, mean age 61.9 ± 16.5 years) referred for CTPA (100 kV) due to suspected pulmonary embolism were randomized in groups of 45 patients each, with either unilateral (A:4 mL/s; B:6 mL/s) or bilateral (C: 6 mL/s; D: 8 mL/s) (Y-shaped line) injection of 50 mL contrast media. Attenuation was assessed including the subsegmental arteries (4th order). Image quality was evaluated by two readers in consensus using a three-point grading scale (3 = excellent image quality, no artifacts, 1 = non-diagnostic). Results Mean pulmonary artery attenuation was significantly higher with bilateral injection (1st to 3rd order: A: 303.6 ± 8.8HU; B: 371.1 ± 11.0HU vs. C: 443.2 ± 24.1HU; D: 562.3 ± 15.3HU, P < 0.001). Evaluation of subsegmental arteries was feasible for all patients in groups B–D, but only in 36/45 (80%) patients in group A. Subsegmental attenuation was significantly higher with bilateral injection (A: 284.7 ± 12.1HU; B: 367.4 ± 12.1HU vs. C: 494.2 ± 21.5HU; D: 562.3 ± 26.7HU, P < 0.001). Image quality was diagnostic for all patients but best for group C (A: 2.15 ± 0.4; B: 2.14 ± 0.5; C: 2.92 ± 0.3, and D: 2.51 ± 0.5). Conclusion Using the same amount of contrast media, bilateral injection yields higher pulmonary artery attenuation and better image quality than unilateral injection. This technique may improve subsegmental pulmonary artery assessment.

Sign in / Sign up

Export Citation Format

Share Document