Clinical presentation, radiological findings and treatment options in Hughes-Stovin syndrome

Abstract

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis associated with arterial or bronchial aneurysms. Even though it was described first in 1911, it is scarcely reported in the literature. Hughes-Stovin syndrome diagnosis is based on clinical manifestations as well as radiological findings. There are no validated criteria or specific laboratory findings to confirm the diagnosis. Computed tomography pulmonary angiography remains the gold standard for the diagnosis and follow-up of radiological findings, as they were recently described in a critical analysis of the largest cohort in the literature. The aim of this review is to draw attention to this rare but potentially fatal disease and to discuss its therapeutic options.

Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre

“…3 Thrombosis is often seen in these aneurysms, and this can lead to embolism and pulmonary hypertension. As proposed by HSS International Study Group (HSSISG) 7 and also summarized by Sanduleanu and Jansen 3 and Kechida et al, 8 the radiological features can be described as (1) early vasculitis-characterized by aneurysmal wall thickening and dilatation as seen in our case 2. (2) Stable aneurysmal diseaseseen as a contrast-filled dilation either as complete aneurysm or pseudoaneurysm in the pulmonary, lobar, segmental, or bronchial artery as seen in our case 3 or (3) unstable aneurysmal disease-seen as signs of extra-luminal leakages of the aneurysm in the form of peri-aneurysmal ground-glass opacities or consolidation with or without loss of aneurysmal wall definition as seen in our case 1.…”

Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement

“…In rare cases, bronchial artery aneurysms or recurrent pulmonary embolism could also be seen [ 56 ]. Typical manifestations of this syndrome are cough, shortness of breath, fever, and chest pain [ 57 ]. As BD, HSS may present with hemoptysis [ 58 ].…”

A Rare but Fatal Behçet Variant: The Hughes–Stovin Syndrome—Successful Case Report and New Evidence from Literature Review

“…A total of 40 patients were analyzed. 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 The comparison of surgical/medical or medical alone therapy on length of follow-up is reported in Fig 3 . No differences in survival rate were observed between the two groups.…”