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Volume 34, Issue 4, December 2023



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Mediterr J Rheumatol 2023;34(4):565-9
Hughes-Stovin Syndrome: A Case Report on a Rare, Life-Threatening Vasculitis
Authors Information

Department of Rheumatology, Pakistan Institute of Medical Sciences, Islamabad, Pakistan

T Khursheed, A Masood , MS Khan, M Sharif, S Shah, MA Miraj

Abstract

Hughes-Stovin Syndrome (HSS) is a rare vasculitic disorder characterised by widespread pulmonary artery aneurysms. It shares some features with Behçet disease. Currently, the diagnosis is based on clinical suspicion. Our case describes a young male who presented with haemoptysis and previous history of pulmonary embolism. Workup was essentially unremarkable, but imaging revealed multiple pulmonary artery aneurysms. Timely initiation of glucocorticoids and immunosuppression with cyclophosphamide led to improvement. High-dose glucocorticoids and immunosuppressants are the mainstays of treatment. Untreated cases can result in fatal outcomes.


Cite this article as: Khursheed T, Masood A, Khan MS, Sharif M, Shah S, Miraj MA. Hughes-Stovin Syndrome: A Case Report on a Rare, Life-Threatening Vasculitis. Mediterr J Rheumatol 2023;34(4):565-9

Article Submitted: 02 Feb 2023; Revised Form: 26 Mar 2023; Article Accepted: 02 Apr 2023; Available Online: 29 Aug 2023

This work is licensed under a Creative Commons Attribution 4.0 International License.

©2023 The Author(s).

https://doi.org/10.31138/mjr.290823.hsr