Multiple pulmonary arterial aneurysms in a young male patient with incomplete Behçet′s syndrome

Kanchinadham, S; Potikuri, Damodaram

doi:10.4103/0970-2113.106121

Cited by 3 publications

(5 citation statements)

References 9 publications

(12 reference statements)

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“…29,68,114 Hematopoietic stem cell transplantation 86 and tumor necrosis factor-α plus infliximab 25 were indicated for patients refractory to conventional immunosuppressive therapy. Indications for interventional embolization included bilateral pulmonary artery involvement, 74 giant pulmonary artery pseudoaneurysm, 87 ruptured PAAs, 64 failed conservative treatment, 34 to avoid potential surgical complications 60 and patients' reluctance to undergo surgical operations.…”

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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“…In our report massive hemoptysis was the cause of death [1,2,[7][8][9]27,33,34,39,47] in 12(21.1%) patients. Early initiation of immunomodulatory drugs, either as single agents or in combination were significantly associated with favorable outcomes and a lower rates of mortality.…”

Section: Discussionmentioning

confidence: 85%

“…3a and c). This radiologic pattern can progress to fatal suffocative hemoptysis [ [1][2][3][4][7][8][9][10][11]27,33,34,39,47]. As such, we consider pulmonary artery pseudoaneurysms (PAPs) to be the most common cause of fatal hemoptysis in HSS.…”

Section: Discussionmentioning

confidence: 99%

“…The study included 57 published HSS patients; 43(75.4%) males and 14(24.6%) females. HSSISG members provided detailed clinical, radiological and treatment data for 41 patients ; while data from other cases were collected by systematic literature review [46][47][48][49]; including earlier case reports [1][2][3][4][7][8][9][10][11]. This systematic literature review was performed by searching Medline/Pubmed and EMBASE using the following keywords (Hughes-Stovin syndrome; pulmonary artery aneurysm in Hughes-Stovin syndrome; pulmonary vasculitis in Hughes-Stovin syndrome).…”

Section: Methodsmentioning

confidence: 99%

“…We included corresponding authors of previously published HSS case reports . Each member provided detailed case data for further analysis, which was combined with other data collected by systematic literature review [46][47][48][49].…”

Section: Introductionmentioning

confidence: 99%

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