Combined pulmonary vasodilator therapy and endovascular intervention for Hughes–Stovin syndrome

Jaramillo, Natalia; Gómez‐Bueno, Manuel; Suárez, Agustín García; López, Alfonso Ortega; Sayago, Inés; Alonso‐Pulpón, Luis; Segovia, Javier

doi:10.1016/j.ijcard.2014.08.091

Cited by 10 publications

(8 citation statements)

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“…Hughes and Stovin [ 1 ] found that the bronchial arteries were significantly affected in their cases, with complete loss of the elastic lamellae, thinning of the media, and distortion of the lumen by conspicuous cushions of edematous sparsely cellular tissue in their autopsy study. Furthermore, BAAs in HSS have also been documented in the recent literature with the use of CTPA [ 15 , 29 ].…”

Section: Discussionmentioning

confidence: 99%

“…In HSS, the underlying cause of intra-cardiac thrombosis is still not clearly understood despite the reporting of multiple cases [ 22 , 32 , 38 ]. In one patient with HSS and widespread in situ thrombosis, an echocardiogram showed tricuspid annular plane systolic excursion (TAPSE 14 mm) and moderate tricuspid regurgitation, with an estimated systolic pulmonary artery pressure of 45 mmHg, all indicative of possible RV dysfunction [ 15 ]. This potential association needs to be further investigated in future studies of HSS-associated intracardiac thrombosis.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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“…Involvement of left hepatic artery aneurysm in HSS patients is rare and reported only by Herb et al 13 Behcet's disease is rarely associated with hepatic artery aneurysm. [14][15][16] Also, extrapulmonary arterial involvement in HSS has been previously reported in the splenic artery, 17 internal iliac artery aneurysm, 18,19 and femoral arteries branches. 12 Optic neuritis, 20 as seen, 2 of our patients, and pan-uveitis, 21 have been previously reported in HSS.…”

Section: Discussionmentioning

confidence: 99%

Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre

Sachdev

Yadav

Balakrishnan

et al. 2023

Clin Med�Insights�Arthritis�Musculoskelet Disord

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“…A total of 40 patients were analyzed. 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 The comparison of surgical/medical or medical alone therapy on length of follow-up is reported in Fig 3 . No differences in survival rate were observed between the two groups.…”

Section: Discussionmentioning

confidence: 99%

A Rare but Fatal Behçet Variant: The Hughes–Stovin Syndrome—Successful Case Report and New Evidence from Literature Review

Ascoli Marchetti,

Belvivere,

Argirò

et al. 2023

Aorta (Stamford)

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Hughes–Stovin syndrome (HSS) is a rare potentially fatal vasculitis supposedly belonging to the spectrum of Behçet disease without ocular involvement. HSS tends to play by a temporal pattern, starting with thrombosis and followed by formation of pulmonary aneurysms. Since its mortality can reach 25% of cases, early recognition and appropriate therapy represent the major clinical challenges. We describe a rare case of HSS successfully treated via multidisciplinary management by an endovascular approach and immunosuppressive therapy.

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Combined pulmonary vasodilator therapy and endovascular intervention for Hughes–Stovin syndrome

Cited by 10 publications

References 5 publications

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

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

Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre

A Rare but Fatal Behçet Variant: The Hughes–Stovin Syndrome—Successful Case Report and New Evidence from Literature Review

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