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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.

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.

Background Anti-TNF alpha treatment may improve endothelial function and mechanical properties of large artery stiffness in patients with inflammatory rheumatic diseases, however studies remain controversial. Objectives To study the effect of TNF alpha blockage with adalimumab for 24 weeks on brachial endothelial function and sub-clinical markers of atherosclerosis in patients with inflammatory rheumatic diseases. Methods A total of 26 patients (14 males, 12 females; mean age, 47±11 years) with inflammatory rheumatic diseases (16, rheumatoid arthritis; 9, ankylosing spondylitis, 1, psoriasic rheumatism), resistant to disease-modifying antirheumatic drugs, were studied before the first infusion of adalimumab and again after 24 weeks of treatment. Endothelial function was assessed by measuring flow mediated dilatation using an ultrasound Doppler technique on the brachial artery. Other markers of sub clinical atherosclerosis were assessed by arterial stiffness via aortic pulse wave velocity, augmentation index and central blood pressure, and carotid intima-media thickness. Data for body mass index, disease activity using DAS-PCR and BASDAI, C-reactive protein, fasting glycaemia, and lipid profile were collected before treatment and after 24 weeks. Results 24 weeks of adalimumab therapy resulted in a reduction of disease activity score and serum levels of C-reactive protein. No significant differences were found in body mass index, total cholesterol, triglycerides and fasting glycaemia. Brachial blood pressure and heart rate remained similar, as well as carotid intima-media thickness. After 24 weeks of treatment with adalimumab, flow mediated dilatation improved significantly; central systolic and diastolic blood pressure decreased significantly; however central pulse pressure, augmentation index and aortic pulse wave velocity remained unchanged. Conclusions In patients with inflammatory rheumatic diseases, treatment with adalimumab for 24 weeks resulted in a significant improvement of endothelial function and central arterial pressure. Disclosure of Interest A. L. Demoux Grant/research support from: Abbvie, K. aissi: None declared, B. chaudier: None declared, S. carijn: None declared, P.-Y. chouc: None declared, H. de baillou: None declared, P. rossi: None declared

Resume Introduction La sarcoidose est une affection granulomateuse systemique de cause inconnue, de localisation principalement mediastinopulmonaire. L’atteinte pericardique et peritoneale est rare, pouvant etre revelatrice de la maladie. Observation Une patiente âgee de 55 ans, etait suivie pour une pericardite recidivante « idiopathique », dont le diagnostic etait remis en cause devant des adenopathies mediastinales et hilaires associees a des images nodulaires pulmonaires. L’exploration par tomographie a emission de positons couplee a la tomodensitometrie mettait en evidence de nombreux foyers hypermetaboliques mediastinopulmonaires et peritoneaux faisant alors evoquer une pathologie carcinomateuse. L’histologie des nodules peritoneaux revelait une sarcoidose. Une corticotherapie etait alors instauree et l’evolution favorable. Conclusion Cette observation montre l’importance d’une explication uniciste chez un patient presentant une pericardite recidivante associee a une atteinte systemique. Bien que rare, la sarcoidose doit etre evoquee et la demarche diagnostique menee jusqu’a la confirmation histologique.

Resume Introduction La maladie de Horton est une vascularite a cellules geantes dont les manifestations inflammatoires ne sont pas uniquement limitees au territoire de l'artere carotide externe. Au travers d'un cas clinique et d'une revue de la litterature, nous proposons l'atteinte pericardique inflammatoire comme une manifestation clinique de cette maladie. Exegese Une femme, âgee de 75 ans, est hospitalisee pour une alteration de l'etat general evoluant depuis trois mois, associee a un febricule, des cephalees frontotemporales et a un important syndrome inflammatoire biologique. La radiographie thoracique revele une cardiomegalie et un aspect compatible avec une pericardite, diagnostic rapidement confirme par l'echocardiographie. La biopsie de l'artere temporale est en faveur d'une maladie de Horton. Une corticotherapie est instauree ; elle permet une regression tres rapide du syndrome inflammatoire et de la pericardite, sans recidive apres six mois de suivi. Conclusion Seule une etude prospective realisant une echographie cardiaque systematique lors du diagnostic d'une maladie de Horton, et cela quels que soient les symptomes cliniques, permettra d'apprecier la prevalence de l'atteinte pericardique et d'evaluer sa valeur pronostique. De meme, la realisation d'une biopsie arterielle temporale semble justifiee devant une pericardite inflammatoire d'allure « idiopathique » subaigue ou chronique du sujet âge. La pathogenie n'est pas connue mais plusieurs hypotheses peuvent etre proposees : orage de cytokines inflammatoires, depot d'immuns complexes, vascularite a cellules geantes des arteres pericardiques ou atteinte inflammatoire interstitielle du pericarde avec ou sans granulomes.

Resume Introduction L'hypereosinophilie peut entrainer des complications cardiaques severes. L'association d'une leucemie aigue lymphoblastique a une hypereosinophilie est rare. Nous rapportons ici le cas d'un patient, âge de 29 ans qui a presente une myocardite necrosante revelant une leucemie aigue lymphoblastique hypereosinophilique. Exegese Notre patient a ete hospitalise dans un tableau d'insuffisance cardiaque, compliquee secondairement d'un accident vasculaire cerebral ischemique en rapport avec un thrombus ventriculaire gauche. Le bilan biologique revelait une hyperleucocytose a 28 500 leucocytes/mm3 avec 18 800 eosinophiles/mm3. Le myelogramme montrait une cytologie de type leucemie aigue lymphoblastique avec une importante eosinophilie et le phenotype etait en faveur de blastes lymphoides de type B. Conclusion Le risque de complications cardiaques majeures d'une hypereosinophilie quelle que soit sa cause doit rendre le praticien vigilant. L'echocardiographie et l'IRM cardiaque pratiquees precocement sont particulierement sensibles pour le depistage de telles complications.

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.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.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.

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.

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.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.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.

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.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.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.