Yasser Emad1, Yasser Ragab2, Melek Kechida3, Aurelien Guffroy4, Michael Kindermann5, Cal Robinson6, Doruk Erkan7, Faten Frikha8, Ossama Ibrahim9, Hamdan Al-Jahdali10, Rafael S Silva11, Leticia Tornes12, Jason Margolesky12, Sami Bennji13, Jung Tae Kim14, Mohamed Abdelbary15, Marianna Fabi16, Maged Hassan17, Vitor Cruz18, Nashwa El-Shaarawy19, Natalia Jaramillo20, Antoine Khalil21, Serkan Demirkan22, Jasna Tekavec-Trkanjec23, Ahmed Elyaski24, B N de FreitasRibeiro25, Issam Kably26, Khalfan Al-Zeedy27, Balakrishnan Jayakrishnan27, Sergio Ghirardo28, Bhupen Barman29, H W Farber30, Sonia Pankl31, Alaa Abou-Zeid32, Pablo Young31, Taoufik Amezyane33, Manoj Kumar Agarwala34, Parag Bawaskar35, Mona Hawass36, Ahmed Saad37, Johannes J Rasker38. 1. Rheumatology Department, Faculty of Medicine, Cairo University, Kasr Al-Ainy St, 11562 Cairo, Egypt. Electronic address: yasseremad68@gmail.com. 2. Radiology Department, Faculty of Medicine, Cairo University, Kasr Al-Ainy St, 11562 Cairo, Egypt. 3. Internal Medicine and Endocrinology Department, Fattouma Bourguiba University Hospital, University of Monastir, Rue du 1er juin 1955, Monastir 5019, Tunisia. 4. Service d'immunologie clinique et médecine interne, centre de référence des maladies auto-immunes systémiques rares (RESO), hôpitaux universitaires de Strasbourg, nouvel hôpital civil, 67091 Strasbourg, France; UFR médecine Strasbourg, université de Strasbourg, 67000 Strasbourg, France. 5. Innere Medizin III (Kardiologie/Angiologie), Universitätskliniken des Saarlandes, Kirrberger Straße, D 66421 Homburg/Saar, Germany. 6. Department of Pediatrics, McMaster University, Hamilton, ON L8N 3Z5, Canada; Department of Paediatrics, Division of Nephrology, The Hospital for Sick Children, Toronto ON, Canada. 7. Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY 10021, USA. 8. Department of Internal Medicine, Hedi Chaker Hospital, 3029 Sfax, Tunisia. 9. Morecambe Bay University Hospitals Lancaster, Lancashire, Ashton Rd, Lancaster LA1 4RP, United Kingdom. 10. Pulmonary Division, Department of Medicine, King Saud University for Health Sciences, King Abdulaziz Medical City, Riyadh 11426, Saudi Arabia. 11. Unidad de Enfermedades Respiratorias, Hospital Regional de Talca, Calle 1 Norte 1990, Talca, Chile. 12. University of Miami Miller School of Medicine, Department of Neurology, Professional Arts Center, 1150 NW 14th St, Suite 609, Miami, FL 33136, United States. 13. Division of Pulmonology, Department of Medicine, Tygerberg Academic Hospital/Stellenbosch University, Francie van Zijl Drive Tygerberg 7505, Cape Town, South Africa. 14. Department of Cardiovascular and Thoracic Surgery, Cheonan Chungmu Hospital, 8 Dagamal 3-gil Seobuk-gu, Cheonan-si, Chungcheongnam-do, Republic of Korea. 15. Department of Radiology, Badr Hospital, Helwan University, 11790 Cairo, Egypt. 16. Pediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy. 17. Chest Diseases Department, Faculty of Medicine, Alexandria University - Al kartoom square, al Azareta, Alexandria 21526, Egypt. 18. Serviço de Reumatologia, Hospital das Clínicas, Faculdade de Medicina, Universidade Federal de Goiás, Goiânia, GO, Brazil. 19. Rheumatology and Rehabilitation Department, Faculty of Medicine, Suez Canal University,Ismailia 4.5 Km the Ring Road, 41522 Ismailia, Egypt. 20. Cardiology Department, Hospital Puerta de Hierro Majadahonda, C/Joaquin Rodrigo 3, Madrid 28222, Spain. 21. Radiology Department, Bichat-Claude Bernard Hospital, HUPNVS, APHP, Paris University, 46 rue Henri Huchard, 74018 Paris, United States of America. 22. Department of Dermatology and Venerology, Izmir Katip Çelebi University Faculty of Medicine, Karabağlar, Izmir, Turkey. 23. Department of Pulmonary medicine, Dubrava University Hospital, Avenija Gojka Šuška 6, 10000 Zagreb, Croatia. 24. Department of prosthesis and orthosis technology Program, Faculty of Applied Health Sciences, Galala University 43511, Suez Governorate, Suez, Egypt. 25. Departamento de Radiologia, Instituto Estadual do Cérebro Paulo Niemeyer, R. do Rezende, 156 - Centro, 20231-092 Rio de Janeiro, RJ, Brazil. 26. Department of Radiology, Section of Vascular and Interventional Radiology, Jackson Memorial Hospital, University of Miami Miller School of Medicine, Miami, FL, USA. 27. Department of Medicine, Sultan Qaboos University Hospital, 123, Al-Khoud, Muscat, Oman. 28. Clinical Department of Medical, Surgical and Health Science, University of Trieste, Piazzale Europa, 1, 34127 Trieste, TS, Italy. 29. Department of General Medicine, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), Mawdiangdiang, Shillong 793018, Meghalaya, India. 30. Tufts University School of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Boston, MA, United States. 31. Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Perdriel 74, C1280 AEB Buenos Aires, Argentina. 32. Public health Department, Faculty of medicine, Cairo University, Kasr Al-Ainy St, 11562 Cairo, Egypt. 33. Department of Internal Medicine, Mohammed V Military Teaching Hospital, Mohammed V-Souissi University, School of Medicine, Rabat, Morocco. 34. Department of Cardiology, Apollo Hospitals, Jubilee Hills, Hyderabad 500096, India. 35. Department of Cardiology, Topiwala National Medical College & B.Y.L Nair Charitable Hospital, Dr. A.L. Nair road, Mumbai 400008, Maharashtra, India. 36. Nephrology Department, El Agouza Police Hospital, El Nil St. Agouza, Giza, Governorate, Egypt. 37. Internal medicine Department, Faculty of Medicine, Cairo University, Kasr Al-Ainy St, 11562 Cairo, Egypt. 38. Faculty of Behavioral, Management and Social Sciences, Department Psychology, Health and Technology, University of Twente, Drienerlolaan 5, 7522NB Enschede, the Netherlands.
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.
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.