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Hughes Stovin Syndrome

Hughes Stovin Syndrome. Morning Report January 19 th , 2010 Jason Kidd. Hemoptysis. Definition: Expectoration of blood from the respiratory tract Massive hemoptysis : >100 ml/24 hours Estimation of blood loss is unreliable. Harrison’s. Hemoptysis : DDx. Airway Disease

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Hughes Stovin Syndrome

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  1. Hughes Stovin Syndrome Morning Report January 19th, 2010 Jason Kidd

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

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

  4. 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

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

  6. Hemoptysis: Evaluation Adapted from Harrison’s

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

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

  9. Kinderman et al

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

  11. 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)

  12. 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 (erythemanodosum) • Positive pathergy test International Study Group for Behcet’s Disease

  13. 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

  14. 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

  15. Treatment • Immunosuppresion • Surgical resection of high risk lesions • Limited to 1 segment of 1 lung • Transcatheterembolization • No specific treatment guidelines

  16. 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

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

  18. 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, ClinRheumatol (2008) 27: 1455-1458 • Harrison’s Internal Medicine • Uptodate.com

  19. Questions?

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