A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). A report by the HSS International Study Group (HSSISG)

Yasser Emad, Yasser Ragab, Melek Kechida, Aurelien Guffroy, Michael Kindermann, Cal Robinson, Doruk Erkan, Faten Frikha, Ossama Ibrahim, Hamdan Al-Jahdali, Rafael S. Silva, Leticia Tornes, Jason Margolesky, Sami Bennji, Jung Tae Kim, Mohamed Abdelbary, Marianna Fabi, Maged Hassan, Vitor Cruz, Nashwa El-ShaarawyNatalia Jaramillo, Antoine Khalil, Serkan Demirkan, Jasna Tekavec-Trkanjec, Ahmed Elyaski, B. N. de FreitasRibeiro, Issam Kably, Khalfan Al-Zeedy, Balakrishnan Jayakrishnan, Sergio Ghirardo, Bhupen Barman, H. W. Farber, Sonia Pankl, Alaa Abou-Zeid, Pablo Young, Taoufik Amezyane, Manoj Kumar Agarwala, Parag Bawaskar, Mona Hawass, Ahmed Saad, Johannes J. Rasker

Research output: Contribution to journalEditorialpeer-review

Abstract

Background: Hughes-Stovin syndrome (HSS) is a systemic disease characterized by widespread vascular thrombosis and pulmonary vasculitis with serious morbidity and mortality. The HSS International Study Group is a multidisciplinary taskforce aiming to study HSS, in order to generate consensus recommendations regarding diagnosis and treatment. Methods: We included 57 published cases of HSS (43 males) and collected data regarding: clinical presentation, associated complications, hemoptysis severity, laboratory and computed tomography pulmonary angiography (CTPA) findings, treatment modalities and cause of death. Results: At initial presentation, DVT was observed in 29(33.3 %), thrombophlebitis in 3(5.3%), hemoptysis in 24(42.1%), and diplopia and seizures in 1 patient each. During the course of disease, DVT occurred in 48(84.2%) patients, and superficial thrombophlebitis was observed in 29(50.9%). Hemoptysis occurred in 53(93.0%) patients and was fatal in 12(21.1%). Pulmonary artery (PA) aneurysms (PAAs) were bilateral in 53(93%) patients. PAA were located within the main PA in 11(19.3%), lobar in 50(87.7%), interlobar in 13(22.8%) and segmental in 42(73.7%). Fatal outcomes were more common in patients with inferior vena cava thrombosis (p = 0.039) and ruptured PAAs (p < 0.001). Death was less common in patients treated with corticosteroids (p < 0.001), cyclophosphamide (p < 0.008), azathioprine (p < 0.008), combined immune modulators (p < 0.001). No patients had uveitis; 6(10.5%) had genital ulcers and 11(19.3%) had oral ulcers. Conclusions: HSS may lead to serious morbidity and mortality if left untreated. PAAs, adherent in-situ thrombosis and aneurysmal wall enhancement are characteristic CTPA signs of HSS pulmonary vasculitis. Combined immune modulators contribute to favorable outcomes.

Original languageEnglish (US)
Pages (from-to)221-229
Number of pages9
JournalInternational Journal of Cardiology
Volume331
DOIs
StateAccepted/In press - 2021

Keywords

  • Behçet's disease
  • Hughes-Stovin syndrome (HSS)
  • Pulmonary artery aneurysms
  • Pulmonary artery pseudoaneurysms
  • Pulmonary vasculitis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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