The Fatal Pulmonary Artery Involvement in Behçet s Disease

The Fatal Pulmonary Artery Involvement in Behçet s Disease Dr. Vedat Hamuryudan Div. Rheumatology, Dept. Internal Medicine Cerrahpasa Medical Faculty, University of Istanbul

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; referred to our clinic

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

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) Maintenance treatment with Azathioprine+Prednisolone

Thorax CT (Jan 2013) Thrombosis in Left descendent PA+ narrowing of Right decendent PA (chronic thrombosis) + peripheral nodules in both lung areas + intracardiac thrombosis

Feb 2013: Bronchial angiography performed because of continuing hemoptysis Enlarged & tortious bronchial arteries in both lung areas along with contrast enhancement of pulmonary arteries

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 Died the same day with abundant hemoptysis.

Final Thorax CT in Sept 2013 showing chronic thrombosed pulmonary arteries and lung hemorrhage there were no pulmonary aneurysms

PAI in BS: Aneurysms & in situ thrombosis of pulmonary arteries & parenchymal lesions Seyahi E, et al. Medicine 2012

Outcome of pulmonary artery involvement (47 patients) 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 Mean follow-up: Survived (n=35)=6.6±2 y Died (n=12)=1.5± 2.3 y Seyahi E, et al. Medicine 2012

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?

Blood circulation in the lungs 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 ) Small anastomoses between PA and BA Murillo H, et al. Semin Ultrasound CT MRI 2012.

Bronchial artery enlargement 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. Enlarged orthotopic bronchial artery in pulmonary thromboembolism Pelage JP. Tech Vasc Intervent Rad 2007 Kalva SP. Tech Vasc Intervent Rad 2009. Image: Yildiz AE et al. Diagn Interv Radiol 2011

Bronchial artery aneurysms Causes: Congenital Behçet s Hughes-Stovin Tuberculosis Sarcoidosis Tumors Hereditary hemorrhagic telangiectasis (Osler-Weber- Rendu disease) Atherosclerosis Idiopathic Restropo CS, et al.semin Ultrasound CT MRI 2012

Bronchial artery aneurysms Diagnosis: Contrast enhanced MDCT angiography with 3-d reconstitution 1,3 Diagnosis & Intervention: Bronchial angiography 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 aneurysm 2

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 (?) 1 Hughes JP & Stovin PG. Br J Dis Chest 1959

Evolution of bronchial artery enlargement in a BS patient with pulmonary artery thrombosis over a period of 16 months Tsai HY, et al. AJR 2011 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

Management of Bronchial artery aneurysms Embolization: Immediate control of bleeding 73-99% Recurrence: 10-55% (depends on the underlying cause) 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) Chun JY, et al. Cardiovasc Intervent Radiol 2010 Kalva SP. Tech Vasc Interventional Radiol 2009 Embolization of BAA in a BS patient (Cerrahpasa)

Bronchial artery enlargement 5 patients: Cerrahpasa Experience The presented case - died 2 underwent embolization 1 complicated by paraplegia both under follow-up 2 under follow-up >8 years; no embolization; small bouts of hemoptysis still present

Conclusions: Bronchial artery enlargement in BS Part of pulmonary vasculitis in BS. Can result in death due to bleeding. Embolization can be life-saving Which patients? Unanswered question: Intensification of immunosuppression?