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Hughes-Stovin Syndrome as an Outcome of Behçet Disease or as a Different Entity

  • Demirkan, Serkan (Department of Dermatology and Venerology, Kirikkale University Faculty of Medicine) ;
  • Gultekin, Yildirim (Department of Cardiovascular Surgery, Kirikkale University Faculty of Medicine)
  • Received : 2017.07.31
  • Accepted : 2017.10.10
  • Published : 2018.02.05

Abstract

Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym "Hughes-Stovin syndrome" was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease.

Keywords

References

  1. Erkan D, Yazici Y, Sanders A, Trost D, Yazici H. Is Hughes-Stovin syndrome Behcet's disease? Clin Exp Rheumatol 2004;22(4 Suppl 34):S64-8.
  2. Hughes JP, Stovin PG. Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest 1959;53:19-27. https://doi.org/10.1016/S0007-0971(59)80106-6
  3. Weintraub JL, DeMayo R, Haskal ZJ, Susman J. SCVIR annual meeting film panel session: diagnosis and discussion of case 1: Hughes-Stovin syndrome. J Vasc Interv Radiol 2001; 12:531-4. https://doi.org/10.1016/S1051-0443(07)61897-1
  4. Grembiale RD, Calderazzo M, Pelaia G, Gallelli A, Roccia F, Vero G. A case of Hughes-Stovin syndrome associated with Budd-Chiari syndrome. Case Rep Clin Pract Rev 2002;3: 92-4.
  5. Khalil A, Parrot A, Fartoukh M, Marsault C, Carette MF. Images in cardiovascular medicine: large pulmonary artery aneurysm rupture in Hughes-Stovin syndrome: multidetector computed tomography pattern and endovascular treatment. Circulation 2006;114:e380-1. https://doi.org/10.1161/CIRCULATIONAHA.106.614636
  6. Mahlo HR, Elsner K, Rieber A, Brambs HJ. New approach in the diagnosis of and therapy for Hughes-Stovin syndrome. AJR Am J Roentgenol 1996;167:817-8.
  7. Reimold WV, Emmrich J, Harmjanz D, Kochsiek K. Multiple aneurysms of the pulmonary artery following recurrent septic pulmonary embolism (Hughes-Stovin syndrome): report of 1 case. Arch Klin Med 1968;215:1-18.
  8. Kapsimali VD, Kanakis MA, Vaiopoulos GA, Kaklamanis PG. Etiopathogenesis of Behcet's disease with emphasis on the role of immunological aberrations. Clin Rheumatol 2010;29:1211-6. https://doi.org/10.1007/s10067-010-1491-6
  9. Kim JT, Oh TY, Chang WH. Rare case of multiple pulmonary artery aneurysms with caval thrombosis: Hughes-Stovin syndrome. Eur J Cardiothorac Surg 2007;31:561-2. https://doi.org/10.1016/j.ejcts.2006.12.005
  10. Ketchum ES, Zamanian RT, Fleischmann D. CT angiography of pulmonary artery aneurysms in Hughes-Stovin syndrome. AJR Am J Roentgenol 2005;185:330-2. https://doi.org/10.2214/ajr.185.2.01850330
  11. Amezyane T, Bassou D, Abouzahir A, et al. Unusual right ventricular thrombus in a woman with Hughes-Stovin syndrome. Intern Med 2010;49:207-8. https://doi.org/10.2169/internalmedicine.49.2941
  12. Kindermann M, Wilkens H, Hartmann W, Schafers HJ, Bohm M. Images in cardiovascular medicine: Hughes-Stovin syndrome. Circulation 2003;108:e156. https://doi.org/10.1161/01.CIR.0000106680.35316.16
  13. Choh NA, Jehangir M, Mir KM, Kuchay S, Wani NA. Hughes-Stovin syndrome: a rare cause of hemoptysis. Lung India 2011;28:285-6. https://doi.org/10.4103/0970-2113.85691
  14. Lee WY, Hoon CS, Kim HR. Massive hemoptysis caused by atypical Behcet's disease. Korean J Thorac Cardiovasc Surg 2014;47:178-80. https://doi.org/10.5090/kjtcs.2014.47.2.178

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  2. A case of Hughes-Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome vol.78, pp.4, 2018, https://doi.org/10.1007/s00393-019-0618-7