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In situ thrombosis in pulmonary arterial aneurysms due to Behçet's disease and efficacy of ımmunosuppressive therapy.
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PMID:  23078955     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
BehçetDisease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers and uveitis, arthritis, and involvement of the gastrointestinal tract, central nervous system and blood vessels. The aneurysms of the pulmonary arteries, with or without thrombosis, are typical manifestation of BD. We report a case with BD, pulmonary arterial aneurysms(PAA) and in situ thrombosis. We aimed to show the effectiveness of immunosuppressive treatment on in situ thrombosis in a case with PAA and BD.
Authors:
Sevket Ozkaya; Unal Sahin; Aziz Gumus; Filiz Taşçı; Halit Cınarka; Asiye Yavuz
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Publication Detail:
Type:  Journal Article     Date:  2012-10-18
Journal Detail:
Title:  Multidisciplinary respiratory medicine     Volume:  7     ISSN:  2049-6958     ISO Abbreviation:  Multidiscip Respir Med     Publication Date:  2012  
Date Detail:
Created Date:  2012-11-05     Completed Date:  2012-11-06     Revised Date:  2013-04-02    
Medline Journal Info:
Nlm Unique ID:  101477642     Medline TA:  Multidiscip Respir Med     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  33     Citation Subset:  -    
Affiliation:
RizeUniversity, Faculty of Medicine, Department of Pulmonary Medicine, Rize, Turkey. ozkayasevket@yahoo.com.
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Journal Information
Journal ID (nlm-ta): Multidiscip Respir Med
Journal ID (iso-abbrev): Multidiscip Respir Med
ISSN: 1828-695X
ISSN: 2049-6958
Publisher: BioMed Central
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Copyright ©2012 Ozkaya et al.; licensee BioMed Central Ltd.
open-access:
Received Day: 27 Month: 6 Year: 2012
Accepted Day: 6 Month: 10 Year: 2012
collection publication date: Year: 2012
Electronic publication date: Day: 18 Month: 10 Year: 2012
Volume: 7 Issue: 1
First Page: 33 Last Page: 33
PubMed Id: 23078955
ID: 3488487
Publisher Id: 2049-6958-7-33
DOI: 10.1186/2049-6958-7-33

In situ thrombosis in pulmonary arterial aneurysms due to Behçet’s disease and efficacy of ımmunosuppressive therapy
Sevket Ozkaya1 Email: ozkayasevket@yahoo.com
Unal Sahin1 Email: drunsahin@yahoo.com
Aziz Gumus1 Email: azizgumus@gmail.com
Filiz Taşçı2 Email: drelifozkaya@hotmail.com
Halit Çınarka1 Email: halitcinarka@hotmail.com
Asiye Yavuz1 Email: dr.elifozkaya@hotmail.com
1RizeUniversity, Faculty of Medicine, Department of Pulmonary Medicine, Rize, Turkey
2RizeEducation and Research Hospital, Department of Radiology, Rize, Turkey

Background

Behçet’s disease (BD) was firstly described by Hulusi Behçet in 1937. It is a systemic vasculitis characterized by recurrent oral and genital ulcers and uveitis, arthritis, and involvement of the gastrointestinal tract, central nervous system and blood vessels [1]. Pulmonary artery aneurysm (PAA) is reported in 1.5 % of adults with BD. Thrombosis of the pulmonary arteries in BD is usually an in situ thrombosis [2]. Some articles reported that immunosuppressive therapy is essential, and anticoagulant therapy might not be required for the treatment of venous disease associated with BD [3]. We aimed to show the effectiveness of immunosuppressive treatment on in situ thrombosis in a case with PAA and BD.


Case presentation

A 25-year-old,non-smoker turkish man was admitted to hospital with complaints of chest pain and cough. The chest radiography showed well-defined, rounded opacities on the left hemithorax (Figure 1). The thorax CT and MRI revealed vascular aneurysms with in situ thrombosis on the left pulmonary artery (Figure 2 and 3). The patient had history of recurrent oral and genital aphthous ulcers. The skin pathergy test was positive (Figure 4). BD was diagnosed based on these findings. The treatment was started with intravenous pulse methylprednisolone (250 mg per day for 3 days) and monthly 1,000 mg cyclophosphamide, and followed by 1 mg/kg per day of methylprednisolone orally, colchium and intravenous pulse of 1,000 mg cyclophosphamidemonthly. Thorax CT was repeated after 2 months of treatment. It showed that PAAs were reduced and in situ thrombosis in PAAs was completely resolved with immunosuppressive treatment (Figure 5). The approval of patient and of institution were taken to use their records for our study.


Conclusions

After the aorta, the pulmonary arteries are the most common site of arterial involvement among the pulmonary manifestations in patients with BD [4]. PAAs associated with BD tend to be multiple, as seen in our patient. The hemoptysis is the commonest symptom of PAA in BD, and one of the leading causes of death [5]. In the present case there was no hemoptysis. The aneurysms of the pulmonary arteries, with or without thrombosis, are typical manifestation of BD [6]. Tunacı et al. reported mural thrombotic changes during regression of PAAs [7]. The underlying pathophysiologic process is inflammation of the vasa vasorum of the tunica media, which causes destruction of the elastic fibers of the media and dilatation of the vessel lumen. Thickening of the vessel wall is caused by inflammation and infiltration by lymphocytes, plasma cells and neutrophils. Thrombosis of the pulmonary arteries in BD is usually an in situ thrombosis [2,8]. Because the main problem is the inflammation of pulmonary arteries, the main stay of treatment should be the anti-inflammatory and immunosuppressive agents in patients with PAA and BD. A combination of cyclophosphamide and methylprednisolone is used most frequently in patients with PAA [9]. We know that anticoagulant therapy could increase the risk of aneurysmal rupture and anticoagulant drugs might be unnecessary in BD [5]. Mehta et al. reported a case with in situ thrombosis with BD [10]. They reported the patient had remained clinically stable with no further episodes of hemoptysis with immunosuppressive treatment including dexamethasone and cyclophosphamide [10]. However, there was no radiologically demonstrated efficacy of immunosuppressive treatment on in situ thrombosis. The aim of this report has been to demonstrate the effectiveness of immunosuppressive treatment on in situ thrombosis with PAA in a patient with BD. Contrast-enhanced thorax CT revealed the in situ thrombosis on the wall of PAAs. After the immunosuppressive treatment the in situ thrombosis was completely resolved and PAAs were reduced.

In conclusion, inflammation in pulmonary arteries is causing in situ thrombosis and it contributes to the development of PAAs in patients with BD. The anti-inflammatory and immunosuppressive drugs are essential for the treatment of in situ thrombosis and PAAs in patients with BD.


Consent

Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Competing interest

The authors declare that they have no competing interests.


Authors’ contributions

SO, US, AG, FT, HC, AY have made substantial contributions to conception and design, or acquisition of data, or analysis and interpretation of data. All authors read and approved the final manuscript.


References
Behçet H,Überrezidivierende, aphtöse, durchein virus verursachtegescwüre am mund, auge und an den genitalenDermatol WochenschrYear: 193710511521157
Yılmaz S,Cimen KA,Pulmonary artery aneurysms in Behçet’s diseaseRheumatol IntYear: 2010301401140310.1007/s00296-009-1092-319693504
Ahn JK,Lee YS,Jeon CH,Koh EM,Cha H-S,Treatment of venous thrombosis associated with Behçet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulationClin RheumatolYear: 20082720120510.1007/s10067-007-0685-z17636362
Yoon YH,Kim KH,Baek WK,Kim JT,Shon KH,Kim YS,et al. Pulmonary artery pseudoaneursym in a patient with Behçet diseaseJ Thorac Cardiovasc SurgYear: 2004127290592
Erkan F,Gul A,Tasalı E,Pulmonary manifestations of Behçet’s diseaseThoraxYear: 20015657257810.1136/thorax.56.7.57211413359
Hiller N,Lieberman S,Chajek-Shaul T,Bar-Ziv J,Shaham D,Thoracic manifestations of Behçet Disease at CTIRadiographicsYear: 20042480180810.1148/rg.24303509115143229
Tunacı M,Ozkorkmaz B,Tunacı A,et al. CT findings of pulmonary artery aneurysms during treatment for Behçet’s diseaseAm J RoentgenolYear: 199917272973310063870
Raz I,Okon E,Chajek-Shaul T,Pulmonary manifestations in Behçet’s syndromeChestYear: 19899558558910.1378/chest.95.3.5852646075
Fresko I,Yurdakul S,Hamuryudan V,et al. The management of Behçet’s syndromeAnn Med InterneYear: 1999150576581
Mehta AA,Jose W,Balamugesh C,Right hilar mass with hemoptysis: An unusual presentation of uncommon disorderLung IndiaYear: 201128430630810.4103/0970-2113.8569822084550

Figures

[Figure ID: F1]
Figure 1 

Chest radiography is showing the well-shaped, round hilar opacities on left hemithorax.



[Figure ID: F2]
Figure 2 

Thorax CT scans are showing the aneurysmatic dilatations and in situ thrombosis in the left pulmonary artery (white arrows).



[Figure ID: F3]
Figure 3 

The thoracic MRI is showing the well-shaped, round hilar opacities on left hemithorax.



[Figure ID: F4]
Figure 4 

The positive pathergy test is seen with pustular lesion on injection area of body (black arrows).



[Figure ID: F5]
Figure 5 

Thorax CT scans showing that in situ thrombosis in PAAs was completely resolved with immunsuppressive treatment (white arrows).



Article Categories:
  • Case Report

Keywords: Behçet’s disease, Pulmonary artery aneurysm, In situ thrombosis, Immunosuppressive treatment.

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