Hughes-Stovin syndrome (HSS): current status and future perspectives

Sanduleanu, Sebastian; Jansen, Tim L.

doi:10.1007/s10067-021-05958-3

Cited by 7 publications

(15 citation statements)

References 9 publications

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“…Hughes-Stovin syndrome is a rare condition, as the largest cohort of patients includes no more than 57 cases [ 1 ]. Thrombophlebitis associated with arterial and/or bronchial aneurysms is the hallmark of the disease [ 2 ].…”

Section: History Of the Diseasementioning

confidence: 99%

“…Chronic leakage is considered as severe disease aggravation resulting in PAP [ 29 ]. The latter will appear on CTPA as an ectatic vascular lumen with sharp and demarcated contrast filling with a perianeurysmal component having a marginal hypodensity [ 1 ].…”

Section: Work-up In Hughes-stovin Syndromementioning

confidence: 99%

“…As the histological findings are based on lymphocytic infiltration, some treatments such as anakinra (anti-IL1 monoclonal antibody), colchicine, anti-tumor necrosis factor α monoclonal antibodies (anti-TNF-α), rituximab (anti- CD20 monoclonal antibody) and tocilizumab (anti-IL-6 antibody) could be discussed [ 1 ]. Anticoagulant therapy is still debated due to a high risk of fatal hemorrhage.…”

Section: Anti-inflammatory Drugs and Anticoagulationmentioning

confidence: 99%

“…Transcatheter embolization may be indicated in a case of an emergency, to stop progressive pulmonary artery aneurysm considered highly likely to rupture without waiting for a response to immunomodulators [ 1 ]. There are several agents used in embolization such as steel coils, Ethibloc and an epoxy, isobutyl cyanoacrylate.…”

Section: Endovascular Treatment (Embolization)mentioning

confidence: 99%

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Clinical presentation, radiological findings and treatment options in Hughes-Stovin syndrome

Kéchida¹,

Daadaa²,

Jomaa³

2022

Reumatologia

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Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis associated with arterial or bronchial aneurysms. Even though it was described first in 1911, it is scarcely reported in the literature. Hughes-Stovin syndrome diagnosis is based on clinical manifestations as well as radiological findings. There are no validated criteria or specific laboratory findings to confirm the diagnosis. Computed tomography pulmonary angiography remains the gold standard for the diagnosis and follow-up of radiological findings, as they were recently described in a critical analysis of the largest cohort in the literature. The aim of this review is to draw attention to this rare but potentially fatal disease and to discuss its therapeutic options.

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Section: History Of the Diseasementioning

confidence: 99%

Section: Work-up In Hughes-stovin Syndromementioning

confidence: 99%

Section: Anti-inflammatory Drugs and Anticoagulationmentioning

confidence: 99%

Section: Endovascular Treatment (Embolization)mentioning

confidence: 99%

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Clinical presentation, radiological findings and treatment options in Hughes-Stovin syndrome

Kéchida¹,

Daadaa²,

Jomaa³

2022

Reumatologia

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“…Hughes–Stovin syndrome (HSS) is another extremely rare systemic vasculitic disease that has also been considered a severe variant of BD. The condition is characterised by widespread deep vein thromboses and pulmonary and/or bronchial artery aneurysms with high morbidity and mortality rates [ 119 ]. In the context of disease severity and a few paediatric case reports [ 120 , 121 ], HSS should also be considered in the young.…”

Section: Mimics Of Bdmentioning

confidence: 99%

Paediatric Behçet’s Disease: A Comprehensive Review with an Emphasis on Monogenic Mimics

Cınar

Romano

Güzel³

et al. 2022

JCM

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Behçet’s disease (BD) is a polygenic condition with a complex immunopathogenetic background and challenging diagnostic and therapeutic concepts. Advances in genomic medicine have provided intriguing insights into disease pathogenesis over the last decade, especially into monogenic mimics of BD. Although a rare condition, paediatric BD should be considered an important differential diagnosis, especially in cases with similar phenotypes. Emerging reports of monogenic mimics have indicated the importance of genetic testing, particularly for those with early-onset, atypical features and familial aggregation. Treatment options ought to be evaluated in a multidisciplinary setting, given the complexity and diverse organ involvement. Owing to the rarity of the condition, there is a paucity of paediatric trials; thus, international collaboration is warranted to provide consensus recommendations for the management of children and young people. Herein, we summarise the current knowledge of the clinical presentation, immunopathogenetic associations and disease mechanisms in patients with paediatric BD and BD-related phenotypes, with particular emphasis on recently identified monogenic mimics.

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Hughes-Stovin-Syndrom: eine lebensbedrohliche Manifestation des Behçet-Syndroms

et al. 2023

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ZusammenfassungDas Hughes-Stovin-Syndrom (HSS) ist eine entzündliche Systemerkrankung unklarer Genese, die inzwischen dem Spektrum des Behçet-Syndroms (BS) zugeordnet wird. Wegweisende Befunde sind rezidivierende Thrombosen des venösen Systems und oberflächliche Thrombophlebitiden in Kombination mit beidseitigen Pulmonalarterienaneurysmen (PAA). Die Pulmonalisangiographie mittels Computertomographie ist von entscheidender diagnostischer Bedeutung, um die (entzündliche) Beteiligung der Pulmonalarterien darzustellen. Die Therapie des HSS orientiert sich an den Empfehlungen der European Alliance of Associations for Rheumatology (EULAR) für das BS und sieht primär eine Immunsuppression mit Cyclophosphamid und Glukokortikoiden vor. Neben einer medikamentösen Therapie sollte eine interventionelle Versorgung der PAA evaluiert werden. Eine spontane PAA-Ruptur muss auch bei Remission der Erkrankung und/oder deutlicher Regredienz des PAA-Durchmessers aufgrund einer fragilen Gefäßarchitektur bedacht werden.

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Hughes-Stovin syndrome (HSS): current status and future perspectives

Cited by 7 publications

References 9 publications

Clinical presentation, radiological findings and treatment options in Hughes-Stovin syndrome

Clinical presentation, radiological findings and treatment options in Hughes-Stovin syndrome

Paediatric Behçet’s Disease: A Comprehensive Review with an Emphasis on Monogenic Mimics

Hughes-Stovin-Syndrom: eine lebensbedrohliche Manifestation des Behçet-Syndroms

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