A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement

Abstract

A case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement Unmasking the true face of a rare syndrome Robinson et al. [16] Case 4: A 21-year-old male; recurrent oral ulcers; no genital ulceration; superficial thrombophlebitis; pulmonary CT angiography a 35 mm right lateral segmental PAA with multiple pulmonary artery in situ thromboses Demirkan and Gültekin [17] Case 5, 6: two patients with HSS who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers Al-Jahdali [18] Case 7: 23-year-old Saudi woman; recurrent oral ulceration; right-lower lobe PAA; papilledema; DVT Yagi et al. [19] Case 8: A 32-year-old male; multiple PAA; DVT of the right leg and the right femoral vein; thrombosis of the vena cava; aphthous ulcer in the oral cavity, an ulcer in the genital region Madiha and Sami [20] Case 9: DVT; oral ulcers; giant aneurysm in the left lower lobe pulmonary artery Summary of HSS case reports without features of the classic triad of BD Hughes and Stovin [1] Case 1, 2: two male patients; segmental PPA with peripheral venous thrombosis Kopp and Green [21] Case 3, 4: two male patients; PAA and recurrent thrombophlebitis Fabi et al. [22] Case 5: A 12-year-old boy; right atrium endocardial mass; jugular vein and cerebral venous thrombosis; deep venous thromboses; PAA Abdelbary et al. [23] Case 6: A 35-year-old Egyptian female lower limb deep vein thrombosis; pulmonary aneurysm Ribeiro et al. [24] Case 7: A 43-year-old male; superficial thrombophlebitis and DVT of the lower limbs; PAA Pankl et al. [25] Case 8: A 41-year-old man; deep venous thrombosis of the right leg, and PAA Al-Zeedy et al. [26] Case 9: A 53-year-old man; DVT and PAA Kably and Reveron [27] Case 10: A 41-year-old male; massive hemoptysis; ruptured PAA; DVT; cardio-venous thromboembolism; pulmonary infarction El Aoud et al. [28] Case 11: A 42-year-old woman; DVT; PAA Jaramillo et al. [29] Case 12: A 47-year-old male; dilated main pulmonary arteries, multiple right bronchial artery aneurysms and a splenic artery aneurysm Silva et al. [30] Case 13: A 25-year-old male; DVT; PAA Grembiale et al. [31] Case 14: DVT; PAA; Budd-Chiari syndrome; a thrombotic occlusion of inferior vena cava Amezyane et al. [32] Case 15: A 28-year-old female; right ventricular thrombus, PAA, iliac vein thrombosis; caval thrombosis Kim et al. [7] Case 16: A 45-year-old man; massive hemoptysis; DVT; bilateral PAA and inferior vena caval thrombosis Chalazonitis et al. [9] Case 17: A 18-year-old, Greek male patient; DVT; PAA; superior sagittal and transverse sinuses Emad et al. [2] Case 18, 19: two male patients; DVT, PAA, superior sagittal sinus thrombosis in one case Balci et al. [33] Case 20: A 41-year-old patient; multiple PAA; thrombus in both the inferior and superior vena cavae Herb et al. [34] Case 21: A 25-year-old man; PAA; multiple aneurysms of the bronchial arteries; severe hemoptysis; aneurysm of the left hepatic artery Margolesky et al. [35] Case 22: A 38-year-old woman; DVT, PAA; right ventricle th...

Pattern of pulmonary vasculitis and major vascular involvement in Hughes-Stovin syndrome (HSS): brief report of eight cases

“…In this report, we describe the pattern of pulmonary vasculitis in a series of patients with HSS. To date, no criteria have been proposed to diagnose HSS; however, it is now established that recurrent thrombophlebitis and/or DVT, PAAs, and intraaneurysmal in situ thrombosis with variable degree of hemoptysis are the constant and key features in patients with HSS otherwise normal coagulation profile [9]. In our report, all patients presented with DVT and other major vein thrombosis.…”

“…In our report, all patients presented with DVT and other major vein thrombosis. Widespread vascular thrombotic events are dominant features in HSS, e.g., vena cava, intra-cardiac, jugular vein, iliac vein, femoral vein, and portal veins [9], and cerebral venous sinus thrombosis (CVST) [7]. Moreover, extensive arterial thrombosis reaching up to the level of the aorta and common iliac and celiac artery aneurysm was recently reported [9].…”

“…lobar, inter-lobar, and segmental, and distributed in both lung fields, and all PAAs are seats of intraaneurysmal in situ thrombosis and mural wall enhancement on post-contrast CTPA images. An important detail that merits consideration in HSS is that the clots in the PA branches in HSS are mostly due to underlying arterial vasculitis rather than venous thromboembolism; the latter fact was explained recently in one patient with superficial thrombophlebitis without actual DVT who developed PAAs and intra-aneurysmal adherent in situ thrombosis [9]. Moreover, the thrombi in the lower extremities in BD and HSS are tightly adherent to the inflamed veins with no tendency for propagation [11].…”

“…For maintenance of remission, we used, besides oral corticosteroids, either azathioprine in a dose of 150 mg/day (in five patients) or pulse cyclophosphamide for at least 1 year duration (in the other three patients). The treatment of pulmonary vasculitis in HSS and BD generally follows the same lines, as these are the only two conditions known to predispose to PAAs [7,9,11].…”

“…Some investigators suggest that HSS is actually a subtype of BD, but we can only speculate about that, as the etiology of both diseases is still uncertain. Such a link seems likely as occasionally HSS has been described as a feature in BD and even as its presenting manifestation [7][8][9]. BD is a systemic vasculitis which can affect venous and arterial vessels of any size.…”