Hughes-Stovin Syndrome: a case report and review of the literature

Chalazonitis, Athanasios; Lachanis, Stefanos; Mitseas, Panagiotis; Argyriou, Panagiotis; Tzovara, Joannie; Porfyrides, Petros; Sotiropoulou, Evangelia; Ptohis, Nikolaos

doi:10.1186/1757-1626-2-98

Cited by 44 publications

(53 citation statements)

References 15 publications

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“…Five case reports, each describing the case of one patient, diagnosed Hughes-Stovin syndrome. 37 21,27,57,81,113,118 and persistent in 2 (11.8%) patients. 18,63 Additionally, one patient was described as having hemoptysis of unknown origin on admission.…”

Section: Resultsmentioning

confidence: 99%

Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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Pulmonary artery aneurysms (PAAs) are the most common type of pulmonary involvement in Behçet' s disease. However, the relationships between clinical features and prognosis have not been sufficiently evaluated. This article describes the results of a comprehensive review, revealing that PAAs have a predilection for hemoptysis manifestations, increased dimensions, right lower lobar location, multiplicity and concurrent intramural thrombus formation. Surgical intervention was needed in one third of patients. Patients with massive hemoptysis and PAA rupture warranted emergency operations. Conservatively treated patients were prone to PAA progression; interventional embolization was associated with higher risks of recurrence and reintervention for PAAs; and surgically treated patients exhibited the highest mortality rates. In conclusion, PAAs in Behçet' s disease are characterized by a predilection for hemoptysis manifestations, right lower lobar location, multiplicity, and concurrent intramural thrombus formation. Both the condition itself and the surgical operations it warrants are linked with high mortality due to PAA hemorrhage.Keywords: hemoptysis; therapeutic embolization; vasculitis. ResumoOs aneurismas das artérias pulmonares (PAA) são as manifestações mais comuns dos pulmões na doença de Behçet. No entanto, as relações entre as características clínicas e o prognóstico ainda não foram devidamente explicadas. O objetivo do presente artigo foi fazer uma ampla revisão da literatura sobre esta questão. As fontes de dados contaram com uma ampla revisão bibliográfica dos anos de 1990 a 2013, sobre os seguintes temas: doença de Behçet, síndrome de HughesStovin, aneurisma de artéria pulmonar e pseudoaneurisma da artéria pulmonar. Os PAA evoluíram com predileção por hemoptise, aumento de dimensões, localização no lobo inferior direito, multiplicidade e formação de trombo intramural. A intervenção cirúrgica foi necessária em um terço dos pacientes. O tratamento cirúrgico emergencial foi indicado na vigência de ruptura do PAA e de hemoptise maciça. Os pacientes tratados conservadoramente evoluíram com propensão para a progressão do PAA. A embolização dos PAA foi associada a uma taxa maior de recidiva e de reintervenção. Houve diferença significativa entre os grupos quanto às taxas de mortalidade, tendo o grupo do tratamento cirúrgico apresentado a maior taxa. Dentre as variáveis citadas, a hemoptise, o envolvimento da artéria lobar e a ruptura do PAA foram fatores preditivos de maior risco de mortalidade. Houve diferenças significativas nas taxas de mortalidade entre os pacientes cirúrgicos e intervencionistas, e entre os três grupos de pacientes: cirúrgico, intervencionista e conservador. Os pacientes tiveram uma sobrevida global de 61,7% em um seguimento médio de 22,5 meses. Os PAA, na doença de Behçet, apresentaram as seguintes predileções: tendência a hemoptise, multiplicidade, localização no lobo inferior direito e presença de trombos intramurais. As rupturas e hemorragias dos PAA, aliadas ao necessário tr...

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Section: Resultsmentioning

confidence: 99%

Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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“…18 In addition, the Hughes-Stovin syndrome, a rare autoimmune disorder of unknown origin that generally affects young adult men, is also characterized by PAA formation, recurrent thrombophlebitis, and a high risk of PAA rupture. 19 However, some authors have suggested that the Hughes-Stovin syndrome is a cardiovascular manifestation of the Behçet syndrome and therefore part of the same disease process. 18,19 PA hypertension (PAH) is an important cause of PAA formation 10,14,20 and has been suggested to be a clinical symptom of an existing PAA.…”

Section: Acquired Causesmentioning

confidence: 99%

Aneurysms of the Pulmonary Artery

Kreibich¹,

Siepe²,

Kroll³

et al. 2015

Circulation

195

294

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“…The distribution of the vascular component of the syndrome is as follows: arterial (7%), venous (25%) or both (68%) [14]. Some authors have proposed that HSS may actually be a partially manifested Behçet's disease (BD) based on their similar findings [12,15,16]. In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms yet most HSS patients do not have typical manifestations of BD such as oral and genital ulcers, uveitis or arthralgia.…”

Section: Discussionmentioning

confidence: 97%