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