Hughes-Stovin syndrome: a case report and literature review
DOI:
https://doi.org/10.18203/2320-6012.ijrms20250266Keywords:
Hemoptysis, Pulmonary artery aneurysms, Huges-Stovin syndromeAbstract
Hughes-Stovin syndrome (HSS) is a rare clinical entity comprised of thrombophlebitis and multiple pulmonary and bronchial aneurysms. Medical literature has documented fewer than 40 cases of HSS. Its pathogenesis and etiology are still unknown. It is considered a variant of Behcet’s disease (BD). HSS is being managed either medically or surgically. We report the case of a 32-year-old Saudi male patient with HSS who initially presented with a pulmonary embolism (PE). Later, he developed hemoptysis that was treated successfully with methylprednisolone. The genetic, etiologic, and pathologic basis of HSS should be explored more in the future to elucidate the disease. Exploration of better and optimal medical and surgical therapeutic regimens is a need for time to treat and prevent morbidity in BD and HSS. There is a dilemma of anticoagulation in these patients that needs to be addressed and investigated properly.
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References
Gupta M, Agrawal A, Iakovou A, Cohen S, Shah R, Talwar A. Pulmonary artery aneurysm: a review. Pulmonary Circulation. 2020;10(1):2045894020908780. DOI: https://doi.org/10.1177/2045894020908780
Kandathil A, Chamarthy M. Pulmonary vascular anatomy and anatomical variants. Cardiovasc Diagn Ther. 2018;8(3):201-07. DOI: https://doi.org/10.21037/cdt.2018.01.04
Samreen I, Darji P, Genobaga S, Doosetty S, Mohta T, Maity G, et al. Pulmonary Artery Aneurysm in Behcet Disease: Medical, Endovascular or Surgical Intervention. Cureus. 2023;15(11):e49368. DOI: https://doi.org/10.7759/cureus.49368
Bu C, Zhang M, Sun Q, Zhang H, Luo J, Liu Q, et al. Pulmonary artery aneurysm: computed tomography (CT) imaging findings and diagnosis. Quant Imaging Med Surg. 20241;14(8):6147-60. DOI: https://doi.org/10.21037/qims-24-462
Giannessi C, Smorchkova O, Cozzi D, Zantonelli G, Bertelli E, Moroni C, et al. Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement. Diagnostics. 2022;12(11):2868. DOI: https://doi.org/10.3390/diagnostics12112868
Park JH, Han MC, Bettmann MA. Arterial manifestations of Behcet disease. AJR. 1984;143:821-25. DOI: https://doi.org/10.2214/ajr.143.4.821
Chalazonitis AN, Lachanis SB, Mitseas P, Argyriou P, Tzovara J, Porfyrides P, et al. Hughes-Stovin Syndrome: a case report and review of literature. Cases J. 2009;(2):98. DOI: https://doi.org/10.1186/1757-1626-2-98
Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. 1999;341:1284. DOI: https://doi.org/10.1056/NEJM199910213411707
Tsai CL, Lu TS, Tsai KC, Chen WJ. Hemoptysis caused by Hughes-Stovin syndrome. Am J Emerg Med. 2005;23:209-11. DOI: https://doi.org/10.1016/j.ajem.2004.05.009
Balci NC, Semelka RC, Noone TC. Multiple pulmonary aneurysms secondary to Hughes-Stovin syndrome: demonstration by MR angiography. J Magn Reson Imaging. 1998;8(6):1323-5. DOI: https://doi.org/10.1002/jmri.1880080620
Ketchum E, Zamanian R, Fleischmann D. CT Angiography of Pulmonary Artery Aneurysms in Hughes-Stovin Syndrome. AJR. 2005;185:330-32. DOI: https://doi.org/10.2214/ajr.185.2.01850330
Remy-Jardin M, Bouaziz N, Dumont P, Brillet PY, Bruzzi J, Remy J. Bronchial and Nonbronchial Systemic Arteries at Multi-Detector Row CT Angiography: Comparison with Conventional Angiography. Radiology. 2004;233:741-9. DOI: https://doi.org/10.1148/radiol.2333040031
Mahlo HR, Elsner K, Rieber A. New approach in the diagnosis of and therapy for Hughes-Stovin syndrome. Am J Roentgenol. 1996;167:817-8. DOI: https://doi.org/10.2214/ajr.167.3.8751710
Lee J, Noh JW, Hwang JW, Kim H, Ahnn JK, Koh EM, et al. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes Stovin syndrome patient. Clin Rheumatol. 2008;27(11):1455-58. DOI: https://doi.org/10.1007/s10067-008-0951-8