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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
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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