Arterial stiffness and stroke in sickle cell disease.

BACKGROUND AND PURPOSE: Large vessels are also affected in sickle cell disease. The aim of this study was to assess several parameters in adult patients with sickle cell disease compared with control subjects and in patients with sickle cell disease with stroke. METHODS: Carotid arterial stiffness, intima-media thickness, and transcranial Doppler ultrasonography were measured. RESULTS: Arterial stiffness and transcranial Doppler velocity were significantly increased in 49 patients with sickle cell disease compared with 47 control subjects (P<0.05) and especially in patients with stroke (P<0.05). CONCLUSIONS: These data suggest that transcranial Doppler and arterial stiffness might be associated to stroke in adult patients with sickle cell disease.

Early atheroma in primary and secondary antiphospholipid syndrome: an intrinsic finding.

OBJECTIVES: The relationship between atherosclerosis and the antiphospholipid syndrome (APS) is unclear. This study compared intima-media thickness (IMT), arterial stiffness, and presence of plaques in APS patients and controls to evaluate the risk of atherosclerosis in this patient population. The study also explored the relationship between these parameters and cardiovascular risk factors. METHODS: Carotid and femoral IMT and stiffness were measured in 58 APS patients and 58 controls. In addition, antiphospholipid antibodies and cardiovascular risk factors were investigated and other systemic lupus erythematosus (SLE)-related serologic parameters were measured. Details of the patients' previous medical history and information regarding disease treatment were analyzed. RESULTS: A significant difference was found between IMT, arterial stiffness, and the presence of plaques in patients and controls (P<0.05). All of these parameters were independent of cardiovascular risk factors. No differences in these parameters were found between patients with primary APS and those with secondary APS, or between patients with thrombosis and those with obstetric manifestations. There was no correlation between SLE disease activity and atheroma. Patients with plaques had taken a lower total dose of corticosteroids and/or hydroxychloroquine. CONCLUSIONS: Some markers of early atherosclerosis could be detected in both primary and secondary APS, irrespective of clinical manifestations. These data suggest that atherosclerosis might be an intrinsic finding in APS patients, independent of cardiovascular risk factors, and that immunosuppressive treatment may prevent atherosclerosis.

Long-term follow-up of hypothenar hammer syndrome: a series of 47 patients.

Hypothenar hammer syndrome (HHS) is an uncommon form of secondary Raynaud phenomenon, occurring mainly in subjects who use the hypothenar part of the hand as a hammer; the hook of the hamate strikes the superficial palmar branch of the ulnar artery in the Guyon space, leading to occlusion and/or aneurysm of the ulnar artery. In patients with HHS, such injuries of the palmar ulnar artery may lead to severe vascular insufficiency in the hand with occlusion of digital artery. To date, only a few series have analyzed the long-term outcome of patients with HHS. This prompted us to conduct the current retrospective study to 1) evaluate the prevalence of HHS in patients with Raynaud phenomenon and 2) assess the short-term and long-term outcome in patients with HHS. From 1990 to 2006, 4148 consecutive patients were referred to the Department of Internal Medicine at the University of Rouen medical center for evaluation of Raynaud phenomenon using nailfold capillaroscopy. HHS was diagnosed in 47 of these 4148 patients (1.13% of cases).Forty-three patients (91.5%) had occupational exposure to repetitive palmar trauma. The more common occupations were factory worker (21.3%), mason (12.8%), carpenter (10.6%), and metal worker (10.6%); the mean duration of occupational exposure to repetitive palmar trauma at HHS diagnosis was 21 years. One patient (2.1%) had recreational exposure (aikido training) to repetitive trauma of the palmar ulnar artery, and 3 other patients (6.4%) developed HHS related to a single direct injury to the hypothenar area. Clinical manifestations were more often unilateral (87.2%) involving the dominant hand (93%). HHS complications included digital ischemic symptoms (ischemia: n = 21, necrosis: n = 20) and irritation of the sensory branch of the ulnar nerve (n = 11). In HHS patients, angiography demonstrated occlusion of the ulnar artery in the area of the Guyon space (59.6%), aneurysm of the ulnar artery in the area of the Guyon space (40.4%), and embolic multiple occlusions of the digital arteries (57.4%). All patients were advised to change their occupational exposure. They were given vasodilators, including calcium channel blocker (n = 37) and buflomedil (n = 12); 36 patients (76.6%) also received oral platelet aggregation inhibitors. Twenty-one patients with digital ischemia/necrosis were further given hemodilution therapy to reduce the hematocrit level to 35%. In 3 patients with HHS-related digital necrosis who exhibited partial improvement with vasodilators, prostacyclin analog therapy (a 5-day regimen of intravenous prostacyclin analog) was instituted, resulting in complete healing of digital ulcer in these 3 patients. Other conservative treatment options included controlling risk factors (smoking cessation, low-lipid diet, therapy for arterial hypertension) and careful local wound care of fingers in the 20 patients with digital necrosis. Only 2 patients, exhibiting digital necrosis and multiple digital artery occlusions, with nonthrombotic ulnar artery aneurysm underwent reconstructive surgery, that is, resection of the aneurysm with end-to-end anastomosis of the ulnar artery. The median length of follow-up in patients with HHS was 15.9 months. Thirteen patients (27.7%) exhibited clinical recurrences of HHS; the median time of HHS recurrence onset was 11 months. Outcome of HHS relapse was favorable with conservative measures in all cases. Awareness of HHS is required to increase suspicion of the disorder so that further exposure to risk factors like repetitive hypothenar trauma can be avoided for these patients; this is of great importance for their overall prognosis. We found favorable outcomes in most patients after conservative measures were initiated; therefore we suggest that surgery may be undertaken in the subgroup of patients who exhibit partial improvement while receiving conservative therapy. Finally, because we observed recurrence of HHS in 27.7% of patients, we note that HHS patients require close follow-up, including both regular and systematic physical vascular examination.

Long-term follow-up of aortic involvement in giant cell arteritis: a series of 48 patients.

To date, only a few series have analyzed the long-term outcome of giant cell arteritis (GCA) patients with aortic involvement, which prompted us to conduct the current retrospective study. Our aims were to 1) determine the prevalence of GCA in patients exhibiting nonatherosclerotic aortic involvement (that is, aortitis, aortic ectasia, and/or aneurysm); and 2) evaluate clinical features and long-term outcome of GCA patients exhibiting aortitis, aortic ectasia, and/or aortic aneurysm.From January 1997 to March 2008, 66 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of nonatheromatous aortic complications (aortitis, aortic ectasia, and/or aneurysm). In these 66 patients, aortic involvement was related to GCA (n = 48), Takayasu arteritis (n = 6), relapsing polychondritis (n = 1), and infection (n = 11).Of the 48 patients with GCA, aortic involvement preceded the initial GCA diagnosis in 1 patient. Aortic involvement was identified in association with GCA in 40 patients (83.3%), and developed after the onset of GCA in the 7 remaining patients (14.6%). Aortic involvement was more often asymptomatic (77.1%). The aortic helical computed tomography (CT)-scan procedure principally showed isolated aortitis (circumferential thickening of the aortic wall >3 mm) in 41 patients (85.4%). In the remaining 7 patients with GCA (14.6%), aortic helical CT scan demonstrated aortic thoracic ectasia and aortitis (n = 3), aortic thoracic aneurysm and both thoracic and abdominal aortitis (n = 3), and both aortic abdominal aneurysm and aortitis (n = 1). All patients were given steroid therapy at a median daily dose of 1 mg/kg initially.At 6-month follow-up, 34 of 48 patients systematically underwent both thoracic and abdominal CT scan. Aortic helical CT scan demonstrated complete disappearance of aortitis in 8.8% of patients, improvement of aortitis in 47.1%, unchanged pattern of aortitis and/or aortic thoracic ectasia/aneurysm in 41.2%, and deterioration of aortic thoracic aneurysm in 1 patient (2.9%). At 18-month follow-up, 11 patients systematically underwent both thoracic and abdominal CT scan. Aortic helical CT scan showed complete disappearance of aortitis (n = 1), improvement of aortitis (n = 1), unchanged pattern of aortic thoracic ectasia/aneurysm (n = 2), and deterioration of aortic thoracic aneurysm (n = 1). At patients' last follow-up, the median daily dose of prednisone was 7 mg. Steroid therapy could be discontinued in 17 patients (35.4%).The current retrospective study suggests that aortic impairment may be more prevalent than previously reported. Our findings suggest that specific inflammatory thickening of the aortic wall is common at the time of GCA diagnosis, and that aortitis may be the first manifestation of GCA-associated aortic complications. Whether isolated aortitis leads to vascular wall injury responsible for late-onset aneurysmal disease remains to be determined. At this time, we recommend long-term monitoring for aortic aneurysms, especially in high-risk subjects, although the optimal frequency and imaging modality have not yet been determined. A yearly screening strategy for thoracic/abdominal aortic aneurysms has been proposed for patients with GCA, including physical examination, 2-view chest radiograph, and abdominal ultrasound.