The Fatal Pulmonary Artery Involvement in Behçet s Disease

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The Fatal Pulmonary Artery

Involvement in Behçet’s Disease

Dr. Vedat Hamuryudan Div. Rheumatology, Dept. Internal Medicine Cerrahpasa Medical Faculty,

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33 years old man

• Sept 2011: Hemoptysis for 4 months + fever,

dyspnea, chest pain

– Thorax CT: Bilateral aneurysms of pulmonary arteries (2.5 cm) +

peripheral nodule + lung hemorrhage + intracardiac thrombosis

• History of ROU, GU & Nodular lesions since 2 years

• Diagnosed as Behçet’s with pulmonary artery

involvement

• Treatment: Cyclophosphamide 1 g/m + 3 pulses of

methylprednisolone + 60 mg/d prednisolone;

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Thorax CT in Sept 2011 showing thrombosed right & left pulmonary aneurysms, peripheral nodule and lung

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Aneurysms regressed but he continued to have

small amounts of hemoptysis

• Thorax CT (Dec 2011) : Regression of PAA

• CT (Apr 2012): Filling defects in the distal and inferior

segments of PA. No aneurysms

• Normal CRP + ESR

– Cyclophosphamide continued (15 pulses in Dec 2012)

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Thorax CT (Jan 2013)

• Thrombosis in Left descendent PA+ narrowing

of Right decendent PA (chronic thrombosis) +

peripheral nodules in both lung areas +

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Feb 2013: Bronchial angiography performed

because of continuing hemoptysis

• Enlarged & tortious bronchial arteries in both lung areas along with contrast enhancement of pulmonary arteries

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Sept 2013 while being on Aza+Pred

• Experienced gross hemoptysis in Sept 2013

• Brought to emergency at another hospital

– Thorax CT: Filling defects of pulmonary arteries &

lung hemorrhage; but no aneurysms.

– Enoxaparine was given with the diagnosis of

pulmonary thromboemboli

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Final Thorax CT in Sept 2013 showing chronic

thrombosed pulmonary arteries and lung hemorrhage

– there were no pulmonary aneurysms

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PAI in BS: Aneurysms & in situ thrombosis of pulmonary

arteries & parenchymal lesions

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Outcome of pulmonary artery involvement (47 patients)

Mean follow-up: Survived (n=35)=6.6±2 y Died (n=12)=1.5± 2.3 y Dead Total (n=47) 12 (26%) PAA (n=34) 9 (26%) PAT (n=13) 3 (23%)

Poor prognostic factors: • Exertional dyspnea

• Large (>3 cm) aneurysm • Pulmonary hypertension

• Delay in diagnosis & treatment

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The presented case

• Aneurysms disappeared

under treatment

– Had normal acute phase responses during follow-up

• But he continued to have

hemoptysis & died with

hemoptysis

• Bleeding from bronchial

arteries?

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Pulmonary arteries: 99% of circulation Bronchial arteries (BA): 1%

Non-bronchial systemic arteries (NBSA) Bronchial arteries:

• Orthotopic= Arise from descending aorta at T5-T6 level (70%)

• Ectopic=From other aortic levels • NBSA= Aortic branches (subclavia,

phrenic, internal mammary, brachiocephalic…)

Blood circulation in the lungs

Murillo H, et al. Semin Ultrasound CT MRI 2012.

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• Changes in pulmonary vascular bed result in enlargement of BA & NBSA.

• obstruction, destruction,

compression

• The enlarged vessels & anastomoses are thin-walled and fragile

• Increased pressure makes them prone to rupture.

• The bronchial arteries are the source

of bleeding in 90% of patients presenting with hemoptysis.

Bronchial artery enlargement

Enlarged orthotopic bronchial artery in pulmonary thromboembolism

Image: Yildiz AE et al. Diagn Interv Radiol 2011 Pelage JP. Tech Vasc Intervent Rad 2007

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Bronchial artery aneurysms

• Causes:

– Congenital – Behçet’s – Hughes-Stovin – Tuberculosis – Sarcoidosis – Tumors – Hereditary hemorrhagic telangiectasis (Osler-Weber-Rendu disease) – Atherosclerosis – Idiopathic…

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Bronchial artery aneurysms

• Diagnosis:

– Contrast enhanced

MDCT angiography with 3-d reconstitution1,3

1_{Wilson SR, et al. AJR 2006} 2_{Ketchum ES, et al. AJR 2005}

3_{Restropo CS, et al.Semin Ultrasound CT MRI 2012}

Three dimensional CT showing prominent and tortuous bronchial arteries surrounding pulmonary artery aneurysm2

• Diagnosis & Intervention:

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Bronchial artery aneurysms in BS

• Hughes & Stovin; 1959

1

_:

– “…pulmonary artery aneurysms may be related to a

qualitative defect, possibly congenital, in the bronchial arteries”

• Possible mechanisms:

– Thrombosis of pulmonary vessels (compromised circulation)

– Increased Pulmonary artery pressure (?)

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Tsai HY, et al. AJR 2011

Evolution of bronchial artery enlargement in a BS patient with pulmonary artery thrombosis over a period of 16 months

Initial image: normal size BA

(arrows) with small distal PA (white arrowhead) & small vasa vasora (black arrowhead)

The same section 16 months later: Enlarged BA (arrows) connecting with enlarged vasa vasora to supply PA

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Embolization:

• Immediate control of bleeding 73-99% • Recurrence: 10-55% (depends on the

underlying cause)

Management of Bronchial artery aneurysms

Complications: (Due to ischemic necrosis of organs supplied by bronchial arteries):

• Spinal cord infarction (1-6%) • Bronchial necrosis

• Esophageal necrosis • Pulmonary infarct

• Cortical blindness (transient)

Embolization of BAA in a BS patient (Cerrahpasa)

Chun JY, et al. Cardiovasc Intervent Radiol 2010 Kalva SP. Tech Vasc Interventional Radiol 2009

The Fatal Pulmonary Artery Involvement in Behçet s Disease