What question did this study set out to answer?

To explore treatment options for a patient with Hughes-Stovin Syndrome and associated complications.

May 20, 2026

A35-06 Hughes-Stovin Syndrome and the Eroding Coil

Key Points

To explore treatment options for a patient with Hughes-Stovin Syndrome and associated complications.
Case report of a 44-year-old man with hemoptysis and leg swelling leading to a diagnosis of HSS.
Interventions included right pulmonary artery coil embolization and ongoing adjustments to immunosuppression and anticoagulation therapy.
Patient was monitored over two years with multiple coil embolizations and recent bronchoscopy for complications.
Patient experienced extensive vascular occlusion and multiple interventions, highlighting the rarity and complexity of HSS treatment.
Coil previously used for embolization was found eroding into a lung lesion, indicating potential life-threatening complications.
Combined immunosuppression emerged as the most supported therapy despite limited evidence.

Abstract

Abstract Intro Hughes-Stovin Syndrome (HSS) is a very rare form of Behçet’s disease involving extensive vascularocclusive disease and pulmonary artery aneurysm (PAA). Morbidity is high, often due to PAA rupture.Determining a treatment plan is difficult, involving immunosuppression, cautious consideration ofanticoagulation therapy, and evaluation for interventional or surgical treatment. Here, we discuss apatient with HSS, who’s extensive treatment course demonstrates the wide range of treatmentoptions for the disease. Case This is a case of a 44-year-old man who who initially presented for five days of hemoptysis and legswelling. He was found to have deep vein thromboses (DVTs) extending throughout bilateral legsthrough his inferior vena cava (IVC), right lower lobe (RLL) PAA and distal PA thrombosis. He wasadmitted and underwent right PAA coil embolization and recannalization of his IVC. Over the nexttwo years, the patient underwent multiple coil embolizations and many adjustments to hismedication regimen, including iterations of combined immunosuppression and numerous trials ofanticoagulation. A recent bronchoscopy revealed an exposed metallic object eroding into the lumenof a cavitary lesion in the right lower lobe (RLL). This is suspected to be the coil placed years earlier.The patient is currently pending surgical resection. Discussion Hughes-Stovin Syndrome poses significant diagnostic and therapeutic challenges to the clinician.The rarity of the disease can make diagnosis difficult. Treatment is even more troublesome, as onemust balance two highly morbid pathologies: extensive occlusive thromboses and large vesselaneurysms. Some guidance can be found from a 2021 critical analysis of 57 HSS cases by Emad et al.,which shows support for treatment with combined immunosuppression. Available evidence neitherstrongly supports or contradicts the routine use of anticoagulation. Other evidence suggests thatthromboses found in pulmonary vasculature most likely arise in situ, rather than representing embolifrom deep veins. Conclusion This case of a 44 year old man with HSS demonstrates the range of treatment options for his veryrare and highly morbid condition. His case shows further uniqueness as the coil embolization thatonce saved his life is now threating it. Little evidence exists to guide treatment decisions, butcombined immunosuppression is the most strongly-supported medical therapy. This abstract is funded by: None

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Cite This Study

Schock et al. (Fri,) studied this question.

synapsesocial.com/papers/6a0d50cdf03e14405aa9cd59 https://doi.org/https://doi.org/10.1093/ajrccm/aamag162.705

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