4. Morning Report January 19 th, 2010 Jason Kidd

5. Hemoptysis Definition: Expectoration of blood from the respiratory tract Massive hemoptysis: >100 ml/24 hours Estimation of blood loss is unreliable Harrison’s

6. Hemoptysis: DDx Airway Disease Inflammatory: bronchitis, bronchiectasis Neoplasms Foreign body Trauma Fistula formation

7. Hemoptysis: DDx cont Pulmonary parenchymal disease Infection: PNA, TB, Aspergilloma, abscess Autoimmune: Goodpasture’s, hemosiderosis, lupus, Wegener’s Genetic: Ehlers-Danlos Cocaine induced Medication induced: Bevacizumab

8. Hemoptysis: DDx cont Pulmonary Vascular Disorders PE A-V malformations Elevated pulmonary pressure (LV failure)

9. Hemoptysis: Evaluation Adapted from Harrison’s

10. Background First described by Hughes and Stovin in 1959 4 cases of multiple pulmonary artery aneurysms associated with venous thrombosis

12. Hughes Stovin Syndrome Mostly men, age 12-40 Present with hemoptysis, cough, dyspnea, chest pain. Can present with fever, elevated ICP

13. Kinderman et al

14. Pathogenesis Unknown Aneurysms thought to be related to weakening of the vessel wall due to inflammation Related to Behcet’s disease?

15. Behcet’s disease Chronic inflammatory disease Manifestations are due to vasculitis Characterized by recurrent oral ulcers and systemic manifestations including: Genital ulcers, ocular disease, skin disease, vascular disease, arthritis More common along ancient silk road Turkey: (80 to 370 per 100,000) Japan, China: (13 to 20 per 100,000) North America: (6 per 100,000)

16. Behcet’s disease: cont No pathognomonic tests, Dx based on clinical findings Criteria for diagnosis Recurrent oral ulcers (3x per year) plus 2 of the following Recurrent genital ulcers Eye lesions (uveitis, retinal vasculitis) Skin lesions (erythema nodosum) Positive pathergy test International Study Group for Behcet’s Disease

18. Behcet’s Disease Vascular disease Involves blood vessels of all sizes Can involve both arterial and venous sides of circulation Large vessel involvement in 1/3 patients Thrombus, stenosis, aneurysm formation Pulmonary arterial involvement in characteristic Hemoptysis is the most common presenting symptom UpToDate

19. Association with Behcet’s? Similarities between the pulmonary involvement of Behcet’s disease (BD) and Hughes-Stovin syndrome (HSS) Gender Mostly young males Common clinical manifestations Fever Arthralgia Thrombosis Pulmonary artery aneurysm (PAA) Association with thrombosis HSS: 100% BD: 80% Common histopathologic findings of PAA: Perivascular inflammation Arterial wall destruction Treatment of choice Cyclophosphamide or azathioprine and corticosteroids Prognosis of HSS and BD (in the presence of PAA) Usually fatal due to the rupture of PAA Adapted from Erkan et al

20. Treatment Immunosuppresion Surgical resection of high risk lesions Limited to 1 segment of 1 lung Transcatheter embolization No specific treatment guidelines

21. Treatment Lee et al describe a case similar to ours 48 yo male p/w fever, dyspnea, wt loss CT showed multiple pulmonary arteries Underwent embolization and later R lobectomy Aneurysms returned, L lobectomy performed Discharged on prednisolone 5 mo later, new aneurysms Started on cytoxan CXR at 3 months  no aneurysms

22. What happened? Received high dose solumedrol x 3 days Had right thoracotomy and lobectomy Path: No active vasculitis Started on Imuran and Prednisone

23. Works Cited Chalazonitis et al, Hughes Stovin Syndrome: a case report and review of the literature, Cases Journal 2009, 2:98 Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 1990; 335:1078 Erkan et al, Is Hughes Stovin Syndrome Behcet’s disease?, Clin Exp Rheumatol 2004; 22(Suppl 34): 564-568 Kinderman M, Wilkens H, Hughes Stovin Syndrome, Circulation 2003;108;e156 Lee et al, Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient, Clin Rheumatol (2008) 27: 1455-1458 Harrison’s Internal Medicine Uptodate.com

24. Questions?