[Systemic sclerosis and macrovascular involvement: Status of the issue in 2019]. / Atteintes macrovasculaires de la sclérodermie : état de la question en 2019.

Systemic sclerosis (SSc) is a rare immune disease leading to fibrosis of the skin and internal organs. Microvasculopathy is a hallmark of SSc. However, some patients have severe macrovascular complications as affecting cerebral, cardiac or peripheral vessels. To date, macrovascular involvement in SSc remains a matter of debate. Many studies have shown an increased prevalence of macrovascular involvement in SSc in comparison with controlled subjects with similar cardiovascular risk factors. Various methods were used: ankle brachial pressure index, intima media thickness, imagery, coronary calcium score, pulse wave velocity, or flow mediated dilation. The pathophysiology of macrovascular involvement remains unknown and is probably multifactorial: accelerated atherosclerosis, endothelial dysfunction, or reflected wave of microvessel obliteration. The aim of this study was to perform a comprehensible review of the literature, through the study of different types of involved vessels. Results of the main studies are summarized in tables according to the method of investigation used.

[Recurrent pericarditis revealing a systemic sarcoidosis]. / Péricardite récidivante révélant une sarcoïdose systémique.

INTRODUCTION: Sarcoidosis is a systemic granulomatous disorder of unknown cause, mainly involving the lung and the mediastinum. Involvement of the pericardium and peritoneum is rare, but can be the first manifestation of the disease. CASE REPORT: A 55-year-old female patient was followed-up for a recurrent "idiopathic" pericarditis. Diagnostis was challenged when she secondarily presented with enlarged hilar and mediastinal lymph nodes associated with pulmonary "nodules". Imaging with (18)F-FDG positron emission tomography with computed tomography showed multiple hypermetabolic foci in the mediastinum and peritoneum, which suggested a malignant disorder. Finally, histopathological evaluation of the peritoneal nodules revealed a sarcoidosis. A corticosteroid therapy was initiated and disease course was favourable. CONCLUSION: This case report highlights the importance of a unique explanation for a patient presenting with recurrent pericarditis associated with a systemic disease. Although rare, sarcoidosis should be discussed and diagnostic procedures should be performed to obtain histological confirmation.

Efficacy of Viabahn in the treatment of severe superficial femoral artery lesions: which factors influence long-term patency?

PURPOSE: To evaluate superficial femoral artery (SFA) occlusive disease treatment by means of covered stents. STUDY DESIGN: retrospective. METHOD: From 2000 to 2005, a Hemobahn/Viabahn endoprosthesis was implanted in 102 limbs (95 patients; mean age: 72.1 years, 52-94) for intermittent claudication (group I, n=50 limbs), critical (group II, n=32) or acute ischemia (group III, n=20). Lesions treated were Trans-Atlantic Inter-Society Consensus (TASC) A (n=9) B (n=42), C (n=28) or D (n=23), associated with a good (2 or 3 leg arteries, n=60) or a poor (1 or 0 artery, n=42) runoff. RESULTS: The endograft was placed successfully in all cases, but 3 early deaths (3.2%) (1 in group II and 2 in group III), and 4 acute thromboses (4%) occurred. Primary and secondary actuarial patency rates were 97+/-1.7%, and 99+/-1% at 1 month, 74+/-4.8% & 84+/-4.1% at 1 year,and 71+/-9.5% & 79+/-8.5% at 3 years, after a mean follow-up of 30.2 months (1-60). Long-term primary and secondary patencies were significantly different between TASC Cand TASC D lesions (P<.004 & .001). CONCLUSION: Severity of lesions, rather than preoperative symptoms or runoff, is mainly to be considered before using Hemobahn/Viabahn endoprosthesis in severe SFA occlusive lesions.

[The ictal bradycardia syndrome: a case report and review of the literature]. / Le syndrome de bradycardie comitiale: cas clinique et revue de la littérature.

Epilepsy is a rare cause of sinus node dysfunction which should be recognised. The authors report the case of a 55 year old woman with refractory epilepsy who had sinus arrest during her epileptic fits. After excluding a possible interaction by long-term Carbamazepine treatment, the diagnosis of the Ictal bradycardia syndrome was made in view of the simultaneous occurrence of severe bradycardia and epileptic activity recorded on electro-encephalography. Sudden death being more common in epilepsy, effective treatment of conductive cardiac abnormalities is essential. The refractory nature of the epileptic fits led to the implantation of a permanent pacemaker in this case.

[Pericarditis: a giant cell arteritis manifestation]. / La péricardite: une manifestation de la maladie de Horton.

INTRODUCTION: Temporal arteritis is a vasculitis in which inflammatory manifestations mainly involve the external carotid artery area but not exclusively. Through a clinical observation and a review of the literature, we suggest that inflammatory pericarditis could represent a manifestation of temporal arteritis. EXEGESIS: A 75-year-old-woman was admitted for progressive physical deterioration which had been evolving for three months, associated with fever, frontotemporal cephalalgia and severe biological inflammatory syndrome. Chest X-ray reveals a cardiomegaly and suggests a pericarditis, which was rapidly confirmed by echocardiogram. Temporal artery biopsy concludes to the diagnosis of a giant cell arteritis. Steroids treatment is prescribed, leading to a rapid regression of the inflammatory state and the pericarditis without relapse after 6 months of follow-up. CONCLUSION: Only prospective studies on systematic echocardiography when faced with the diagnosis of giant cell arteritis, whatever clinical symptoms, will enable to appreciate the prevalence and prognosis value of this manifestation. Moreover, temporal artery analysis seems to be justified when faced with a sub-acute or chronic "idiopathic" inflammatory pericarditis occurring in the elderly patient. Physiopathogeny is unknown but some hypothesis can be proposed: inflammatory cytokines storm, immune complexes deposition, giant cell vasculitis of pericardial arteries or inflammatory interstitial lesion of the pericardium with or without granuloma.

[Necrotic myocarditis in acute eosinophilic lymphoblastic leukaemia]. / Myocardite nécrosante révélant une leucémie aiguë lymphoblastique hyperéosinophilique.

INTRODUCTION: Hypereosinophilia can cause severe cardiac complications. The association between an acute lymphoblastic leukemia and hypereosinophilia was rare. We report a case of a 29-year-old man who presented a heart failure secondary to necrotic myocarditis related to an acute eosinophilic lymphoblastic leukaemia. EXEGESIS: The patient developed a heart failure and secondary a cardio-embolic stroke, due to a large mobile left ventricle thrombosis. His peripheral blood showed a total white count of 28,500 leucocytes/mm3 with 18,800 eosinophils/mm3. The myelogram cytology showed precursor B-cell acute lymphoblastic leukaemia with hypereosinophilia. CONCLUSION: The possibility of the rapid emergence of cardiac lesions in hypereosinophilic syndromes warrants very close physician vigilance. An Echocardiography and MRI performed at the early stage and in the follow-up allow to detect and to manage these cardiac disorders.

Efficacy of Hemobahn in the treatment of superficial femoral artery lesions in patients with acute or critical ischemia: a comparative study with claudicants.

PURPOSE: To assess the results of covered stents in the treatment of superficial femoral artery (SFA) occlusive disease. METHOD: From July 2000 till June 2003, 32 patients (34 limbs) were scheduled for procedures including Hemobahn deployment in the SFA. Indication for treatment was claudication (group I, N=15 patients and 16 limbs, 31.2% occlusions) or critical and acute ischemia (group II, N=17 patients and 18 limbs, 61.1% occlusions). TASC D SFA lesions were excluded. No limb artery was patent pre-operatively in 19% and 89% of limbs in groups I and II, respectively (p=0.00001). RESULTS: Outflow procedures were performed simultaneously in one limb in group I and 12 in group II (p=0.0003). The technical, hemodynamic and clinical success rates were 100, 100 and 94.1%, respectively. Mean follow-up was 18.1 months. Primary patency rates at 12 months were 81.3+/-10.6% in group I and 88.6+/-9.0% in group II (p=0.547). At 12 months, the secondary patency and limb salvage rates were, respectively, 87.5+/-8.9 and 100% in group I and 87.5+/-8.93 and 94.45+/-6.71% in group II. CONCLUSION: Treatment of SFA occlusive lesions (excluding TASC D lesions) with the Hemobahn covered stent yielded good results for both claudicants with good outflow and patients with critical or acute ischemia with bad outflow, if concomitant outflow-improving procedures were performed.

Endovascular treatment of iliocaval occlusion due to idiopathic retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis is a rare cause of venous occlusion that is mainly treated conservatively or surgically. However, endovascular techniques can be proposed for its treatment. We report here a case of a 64-year-old man who was admitted for bilateral lower limb swelling due to iliocaval occlusion caused by idiopathic retroperitoneal fibrosis. Due to high anesthetic risk, related mainly to congestive heart failure, the patient was treated percutaneously by balloon angioplasty and stenting instead of receiving surgical treatment. Clinical and radiological results were excellent, both early and in midterm, confirming that endovascular techniques constitute an interesting alternative to surgery, especially in fragile patients.