Pulmonary embolism versus pulmonary vasculitis in Hughes-Stovin syndrome: Characteristic computed tomography pulmonary angiographic findings and diagnostic and therapeutic implications. HSS International Study Group

Yasser Emad*, Yasser Ragab, Harrison W. Farber, Doruk Erkan, Ossama Ibrahim, Michael Kindermann, Jasna Tekavec-Trkanjec, Balakrishnan Jayakrishnan, Nashwa El-Shaarawy, Melek Kechida, Pablo Young, Sonia Pankl, Marianna Fabi, Parag Bawaskar, Issam Kably, Sergio Ghirardo, Faten Frikha, Alaa Abou-Zeid, Maged Hassan, Cal RobinsonMohamed H. Abdelbary, Leticia Tornes, Jason Margolesky, Bhupen Barman, Sami Bennji, Manoj kumar Agarwala, Khalid Alhusseiny, Taoufik Amezyane, Rafael S. Silva, Vitor Cruz, Bruno Niemeyer, Khalfan Al-Zeedy, Hamdan Al-Jahdali, Natalia Jaramillo, Serkan Demirkan, Aurelien Guffroy, Jung tae Kim, Nikolas Ruffer, Samar Tharwat, Diletta Cozzi, Mabrouk Abdelali, Tubig c. Joy, Mona Sayed, Juljani Sherwina, Tamer Gheita, Johannes j. Rasker

*Corresponding author for this work

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Abstract

Background and aim: Hughes-Stovin syndrome (HSS) is a rare systemic vasculitis with widespread venous/arterial thrombosis and pulmonary vasculitis. Distinguishing between pulmonary embolism (PE) and in-situ thrombosis in the early stages of HSS is challenging. The aim of the study is to compare clinical, laboratory, and computed tomography pulmonary angiography (CTPA) characteristics in patients diagnosed with PE versus those with HSS. Methods: This retrospective study included 40 HSS patients with complete CTPA studies available, previously published by the HSS study group, and 50 patients diagnosed with PE from a single center. Demographics, clinical and laboratory findings, vascular thrombotic events, were compared between both groups. The CTPA findings were reviewed, with emphasis on the distribution, adherence to the mural wall, pulmonary infarction, ground glass opacification, and intra-alveolar hemorrhage. Pulmonary artery aneurysms (PAAs) in HSS were assessed and classified. Results: The mean age of HSS patients was 35 ± 12.3 years, in PE 58.4 ± 17 (p < 0.0001). Among PE 39(78 %) had co-morbidities, among HSS none. In contrast to PE, in HSS both major venous and arterial thrombotic events are seen. Various patterns of PAAs were observed in the HSS group, which were entirely absent in PE. Parenchymal hemorrhage was also more frequent in HSS compared to PE (P < 0.001). Conclusion: Major vascular thrombosis with arterial aneurysms formation are characteristic of HSS. PE typically appear loosely-adherent and mobile whereas “in-situ thrombosis” seen in HSS is tightly-adherent to the mural wall. Mural wall enhancement and PAAs are distinctive pulmonary findings in HSS. The latter findings have significant therapeutic ramifications.

Original languageEnglish
Article number109040
JournalThrombosis Research
Volume239
DOIs
Publication statusPublished - 1 Jul 2024

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