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Hughes–Stovin syndrome: is it incomplete Behçet’s?

Report of two cases and review of the literature

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Abstract

In this report, we described two male patients with Hughes–Stovin syndrome. The first patient was a 26-year-old male who initially presented with deep vein thrombosis (DVT) in the right lower limb followed shortly by hemoptysis. Pulmonary CT angiography revealed bilateral pulmonary artery aneurysms secondary to underlying pulmonary vasculitis. While the second case was a 16-year-old male patient who initially presented with generalized fits due to sagittal sinus thrombosis and later followed by DVT in the left lower limb. Pulmonary CT angiography showed small pulmonary artery aneurysms with thrombosis of peripheral pulmonary artery branches. Both patients were treated successfully with steroids and immunosuppressive therapy and showed clinical improvement.

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References

  1. Hughes JP, Stovin PG (1959) Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest 53:19–27

    Article  PubMed  CAS  Google Scholar 

  2. Weintraub JL, DeMayo R, Haskal ZJ, Susman J (2001) SCVIR annual meeting film panel session: diagnosis and discussion of case 1: Hughes–Stovin syndrome. J Vasc Interv Radiol 12:531–534

    Article  PubMed  CAS  Google Scholar 

  3. Durieux P, Bletry O, Huchon G et al (1981) Multiple pulmonary arterial aneurysms in Behcet’s disease and Hughes–Stovin syndrome. Am J Med 71:736–741

    Article  PubMed  CAS  Google Scholar 

  4. Grenier P, Bletry O, Cornud F, Godeau P, Nahum H (1981) Pulmonary involvement in Behcet disease. AJR Am J Roentgenol 137:565–569

    PubMed  CAS  Google Scholar 

  5. Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D (2004) Thoracic manifestations of Behçet disease at CT. Radiographics 24:801–808

    Article  PubMed  Google Scholar 

  6. Greene RME, Saleh A, Taylor AKM et al (1998) Non-invasive assessment of bleeding pulmonary artery aneurysms due to Behçet disease. Eur Radiol 8:359–363

    Article  PubMed  CAS  Google Scholar 

  7. Ketchum ES, Zamanian RT, Fleischmann D (2005) CT angiography of pulmonary artery aneurysms in Hughes–Stovin syndrome. AJR Am J Roentgenol 185:330–332

    PubMed  Google Scholar 

  8. Durieux P, Bletry O, Huchon G, Wechsler B, Chretien J, Godeau P (1981) Multiple pulmonary arterial aneurysms in Behçet’s disease and Hughes–Stovin syndrome. Am J Med 71:736–741

    Article  PubMed  CAS  Google Scholar 

  9. Erkan F, Gul A, Tasali E (2001) Pulmonary manifestations of Behçet’s disease. Thorax 56:572–578

    Article  PubMed  CAS  Google Scholar 

  10. Erkan F (1999) Pulmonary involvement in Behçet’s disease. Curr Opin Pulm Med 5:314–318

    Article  PubMed  CAS  Google Scholar 

  11. Kindermann M, Wilkens H, Hartmann W, Schäfers HJ, Böhm M (2003) Images in cardiovascular medicine. Hughes–Stovin Syndrome. Circulation 108:e156

    Article  PubMed  Google Scholar 

  12. Erkan D, Yazici Y, Sanders A, Trost D, Yazici H (2004) Is Hughes–Stovin syndrome Behcet’s disease? Clin Exp Rheumatol 22(4 Suppl 34):S64–S68

    PubMed  CAS  Google Scholar 

  13. Nishi K, Myou S, Ooka T, Taitou H, Fujimura M, Matsuda T (1993) A case of Behcet’s disease presenting with Hughes–Stovin syndrome. Nihon Kyobu Shikkan Gakkai Zasshi 31(3):379–384

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University, Cairo, Egypt

    Yasser Emad & Tamer Gheita

  2. Rheumatology and Rehabilitation Department, Dr. Erfan and Bagedo Hospital, King Fahad Street, P.O. Box 6519, Jeddah, 21452, Saudi Arabia

    Yasser Emad

  3. Radio Diagnosis Department, Faculty of Medicine, Cairo University, Cairo, Egypt

    Yasser Ragab

  4. Radio Diagnosis Department, Dr. Erfan and Bagedo Hospital, Jeddah, Saudi Arabia

    Yasser Ragab

  5. Neurology Department, Dr. Erfan and Bagedo Hospital, Jeddah, Saudi Arabia

    Abd El-Hakim Shawki

  6. General Surgery Department, Faculty of Medicine, Cairo University, Cairo, Egypt

    Ahmed El-Marakbi

  7. Vascular Unit, Dr. Erfan and Bagedo Hospital, Jeddah, Saudi Arabia

    Ahmed El-Marakbi

  8. Rheumatology Department, Faculty of Medicine, El Azhar University, Cairo, Egypt

    Mohamed Hanfi Salama

Authors
  1. Yasser Emad
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  2. Yasser Ragab
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  3. Abd El-Hakim Shawki
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  4. Tamer Gheita
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  5. Ahmed El-Marakbi
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  6. Mohamed Hanfi Salama
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Corresponding author

Correspondence to Yasser Emad.

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Emad, Y., Ragab, Y., Shawki, A.EH. et al. Hughes–Stovin syndrome: is it incomplete Behçet’s?. Clin Rheumatol 26, 1993–1996 (2007). https://doi.org/10.1007/s10067-007-0609-y

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  • DOI: https://doi.org/10.1007/s10067-007-0609-y

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