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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

Hughes Stovin Syndrome

Morning Report

January 19th, 2010

Jason Kidd


Hemoptysis
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
Hemoptysis: DDx

  • Airway Disease

    • Inflammatory: bronchitis, bronchiectasis

    • Neoplasms

    • Foreign body

    • Trauma

    • Fistula formation


Hemoptysis ddx cont
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


Hemoptysis ddx cont1
Hemoptysis: DDx cont

  • Pulmonary Vascular Disorders

    • PE

    • A-V malformations

    • Elevated pulmonary pressure (LV failure)


Hemoptysis evaluation
Hemoptysis: Evaluation

Adapted from Harrison’s


Background
Background

  • First described by Hughes and Stovin in 1959

    • 4 cases of multiple pulmonary artery aneurysms associated with venous thrombosis


Hughes stovin syndrome1
Hughes Stovin Syndrome

  • Mostly men, age 12-40

  • Present with hemoptysis, cough, dyspnea, chest pain.

  • Can present with fever, elevated ICP


Kinderman et al


Pathogenesis
Pathogenesis

  • Unknown

  • Aneurysms thought to be related to weakening of the vessel wall due to inflammation

  • Related to Behcet’s disease?


Behcet s disease
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)


Behcet s disease cont
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


Behcet s disease1
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


Association with behcet s
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


    Treatment
    Treatment

    • Immunosuppresion

    • Surgical resection of high risk lesions

      • Limited to 1 segment of 1 lung

    • Transcatheterembolization

    • No specific treatment guidelines


    Treatment1
    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


    What happened
    What happened?

    • Received high dose solumedrol x 3 days

    • Had right thoracotomy and lobectomy

    • Path: No active vasculitis

    • Started on Imuran and Prednisone


    Works cited
    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



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