Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide\u2014a report by the HSS International Study Group

Yasser Emad,Jung Tae Kim,Rafael S Silva,Manoj Kumar Agarwala,Marianna Fabi,M Kéchida ,Maged Hassan,Pablo Young,Cal Robinson,Bruno Niemeyer,F Frikha ,Mohamed Abdelbary ,Khalfan Al-Zeedy,Bhupen Barman,Leticia Tornes,Sonia Pankl,Vítor Alves Cruz ,Sergio Ghirardo,Natalia Jaramillo,Jasna Tekavec-Trkanjec,I Kably ,Yasser Ragab,B Jayakrishnan ,Sami Bennji,Ahmed Saad,Nashwa El-Shaarawy,Omer H M Ibrahim ,Pramodini Himmatrao Bawaskar ,Aurélien Guffroy ,Jason Margolesky,A A Abou‐Zeid ,Michael Kindermann,Hamdan Al-Jahdali,T Amézyane ,Serkan Demirkan,Doruk Erkan,Harrison W Farber,Johannes J Rasker

doi:10.1007/s10067-021-05912-3

Abstract

IntroductionHughes-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.ObjectivesThe 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.MethodsThe 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.ResultsThis 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.ConclusionThe 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.

Highlights

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
Due to a lack of diagnostic criteria for HSS, in previous reports, diagnosis of HSS was based on the presence of typical disease features such as (a) widespread recurrent vasculo-occlusive disease in the form of recurrent superficial thrombophlebitis, deep venous thrombosis (DVT), cerebral venous sinus thrombosis (CVST), intracardiac thrombosis, or arterial thrombosis; (b) pulmonary vasculitis with typical computed tomography pulmonary angiography (CTPA) features, notably pulmonary artery aneurysms and in situ thrombosis and/ or bronchial artery aneurysms; and (c) normal coagulation profiles
After extensive review of available CTPA images, a final consensus was reached by HSS International Study Group (HSSISG) members regarding the radiological definitions of each individual lesion

Summary

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. The Hughes-Stovin syndrome (HSS) was named after two British physicians in 1959 John Patterson Hughes and Peter George Ingle Stovin They described two male patients with systemic illness, recurrent deep venous thrombosis (DVT), and segmental pulmonary artery aneurysms (PAA) with recurrent hemoptysis [1]. Similar clinical associations were observed in 1912 by Beattie and Hall, who described a previously healthy man who died suddenly from profuse suffocative hemoptysis [2].Upon autopsy, an aneurysm was found to have ruptured into the adjacent lung tissue, ulcerating in the main branch of the right bronchus and bulging into the right side of the pericardium These findings indicated “extraluminal” extension of this inflammatory process [2]

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