[Upper-extremity venous thrombosis: A retrospective study about 160 cases]. / Épidémiologie des thromboses veineuses des membres supérieurs : étude rétrospective de 160 thromboses aiguës.

AIM: To describe the clinical features and etiologies of upper limb venous thrombosis (ULVT). METHODS: All patients with a clinically suspected ULVT, were included retrospectively from January to December 2016. Diagnosis of ULVT was based on doppler-ultrasonography. Clinical features, topography and symptomatic pulmonary embolism (PE) were analyzed. The sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative value (NPV) of clinical symptoms leading to ULVT suspicion were estimated by comparing patients with and without ULVT. RESULTS: Among 488 patients with a suspected ULVT, 160 were diagnosed with ULVT, including, 80 with deep venous thrombosis (DVT) and 80 with superficial venous thrombosis (SVT). Symptomatic PE was found in 2.5 % of cases (n=4). None of the clinical symptoms of ULVT had a sensitivity greater than 40 %. For DVT, presence of superior vena cava syndrome had a 100 % PPV, 71.6 % NPV and 100 % Sp. For SVT, the presence of an cord-like induration had a 85.7 % PPV, 75.3 % NPV and 98.4 % Sp. An endovenous device was present in 87.5 % of DVT and 97.5 % of SVT cases. Malignant hemopathy was found in 43.8 % and 31.3 % of cases of DVT and SVT, respectively. Sepsis and solid neoplasia were present in 25 % and 15 % of cases of ULVT, respectively. Peripherally inserted central catheter or implantable sites were present in 40 % and 17.5 % of DVT patients. No solid neoplasia, hematological malignancy or thrombophilia were diagnosed in patients with ULVT. CONCLUSION: An endovenous device was involved in 92.5 % of cases of ULVT. The prevalence of symptomatic PE was low. Hematological malignancies, sepsis and neoplasia were the most common conditions present in patients with ULVT.

[Late-onset systemic sclerosis: A retrospective study of 27 patients diagnosed after the age of 70]. / Sclérodermie systémique de révélation tardive : étude rétrospective de 27 patients diagnostiqués après l'âge de 70 ans.

BACKGROUND: The aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70. PATIENTS AND METHODS: This is a retrospective study of patients aged above 70 years at the time of diagnosis of SSc and followed at an internal medicine unit between 2000 and 2015. Co-morbidities and clinical characteristics were analyzed, as well as survival at 1, 2 and 3 years. RESULTS: Of 246 patients, 27 (11%) were included (89% women, 96% Caucasians, age 78.3±4.5 years). Synchronous cancer was noted in 3 patients. SSc was mostly limited cutaneous only (24/27), with telangiectasia (63%), gastroesophageal reflux (59%) and digital ulcers (22%), and was associated with anti-centromere antibody (69%). Interstitial lung disease was not frequent (29%). Pulmonary arterial hypertension (PAH) was suspected at diagnosis of SSc in 14 cases (52%), but only 5 patients had undergone heart catheterization, with severe PAH in 3 cases. Survival at 1 and 3 years was 85.2% and 66.7%, and was worse in the case of suspected PAH, at 78.6% and 57.1% respectively. CONCLUSION: Cases of SSc diagnosed after 70 years are mostly limited cutaneous forms. Suspicion of PAH is frequent, and PAH may be the main initial sign of the disease for patients at this age. There may be association with synchronous cancer. Survival is poor.

Radiographic presentation of musculoskeletal involvement in Werner syndrome (adult progeria).

Werner syndrome (i.e., adult progeria) is a rare autosomal recessive disorder caused by mutations of the WRN gene, which is characterized by the premature appearance of features associated with normal aging and cancer predisposition. Patients with Werner syndrome can present with musculoskeletal complaints, associated with suggestive radiographic features with a potential prognostic or therapeutic impact. This review illustrates the main radiographic features of Werner syndrome, focusing on the musculoskeletal system, such as soft-tissue calcification, muscular atrophy, osteoporosis, foot deformities, osteitis and osteomyelitis, and bone or soft-tissues malignancies. The identification of these features by radiologists can therefore be useful in the clinical screening of Werner syndrome.

[Qualitative and quantitative evaluation of an internal medicine assistance line dedicated to the diagnosis and treatment of diseases for general practice]. / Évaluation qualitative et quantitative d'un service d'assistance téléphonique de médecine interne dédié à l'aide diagnostique et thérapeutique de pathologies relevant du champ de la médecine générale.

PURPOSE: Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided. METHODS: A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line. RESULTS: Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n=93), with a mean age of 46 years, graduated from Nantes University (65%, n=86), practicing ambulatory general medicine (69%, n=164) in Loire-Atlantique department area (82%, n=176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n=166) concerning clinical issues (57%, n=155). A sole telephone advice was the main type of answer provided (56%, n=147) and a visit to the emergency room was prevented for 17% of calls. CONCLUSION: The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered.

Cold climate could be an etiologic factor involved in Raynaud's phenomenon physiopathology. Epidemiological investigation from 954 consultations in general practic.

AIM: The physiopathology of Raynaud's phenomenon (RP) is not currently fully resolved. The cold seems to be not only an important factor triggering attacks, but also inducing RP. The aims of this study were to assess the prevalence of RP in Nantes urban district, and study the relationship between RP prevalence and cold climate. METHODS: Patients aged between 10 and 80 years old, consulting in five Nantes General Practices, from June 2011 and March 2012, were included. Patients presenting RP underwent a full clinical examination. Subjects not meeting Allen and Brown criteria benefited from at least a dosage of Anti-Nuclear Antibodies and a naifold Capillaroscopy. Climate data provided by French national weather agency allowed establishing an average of observed temperatures during the past five years and correlating them to the observed prevalence. RESULTS: Of 954 patients included, 78 had a RP, for an overall prevalence estimated at 8.2%. The prevalence among women (8.9%) was slightly higher than men (7.3%). Secondary form represented 5.1% of RP. In the RP group, 13 patients were active smokers, mean BMI was 22.3±3.2 kg/m², and only 4 patients were treated by vasoconstrictor therapy. According to French national weather agency, between 2007 and 2011, mean temperature of January in Nantes area was 5.8 °C. CONCLUSION: We confirmed that the lower winter temperatures a region experiences, the higher the prevalence of RP, thus raising the question of the physiopathological role of the cold in the induction or in the revelation of RP.

[Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation working group of the Société française de médecine vasculaire]. / Bilan étiologique minimal du phénomène de Raynaud : un consensus d'experts.

About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.

[Superior vena cava syndrome induced by pacemaker probes, 12 years after introduction]. / Un syndrome cave supérieur 12ans après l'implantation d'un pacemaker.

Superior vena cava syndrome is a rare disease, most often found to result from a malignant process, which causes extrinsic compression of the superior vena cava. In recent years, there has been an increase of superior vena cava syndrome related to medical devices (implantable site, pacemaker [PM], central venous line for parenteral nutrition...). We report the case of a 37-year-old patient who developed a superior vena cava syndrome 12 years after implantation of a PM. The diagnosis was established on venography after two negative venous-CT focused on the superior vena cava. The superior vena cava syndrome improved immediately after angioplasty and stenting covering the PM probes at the superior vena cava/brachiocephalic venous trunk junction.

[Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation Working Group of the French Vascular Medicine Society]. / Bilan étiologique minimal du phénomène de Raynaud : un consensus d'experts.

About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.

[Orofacial manifestations of systemic sclerosis: A study of 30 consecutive patients]. / Les manifestations buccofaciales de la sclérodermie systémique : étude de 30 patients consécutifs.

INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87 %, mean age 58.6 + or - 13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33 % of cases), or osteolytic lesions (7 %) is poorly known.

[Necrotizing hand-foot skin reaction induced by antiangiogenic in a patient with Thevenard neuroacropathy]. / Syndrome pied-main nécrotique sous antiangiogéniques chez une patiente porteuse d'une acropathie ulcéromutilante de Thévenard.

Antiangiogenic agents are an innovative oral chemotherapy prescribed in metastatic renal cancer and gastrointestinal stromal tumors (GIST). These molecules have several side effects. A woman, with moderate hypertension and severe Thevenard's ulceromutilating acropathy, presented renal cancer with lung metastasis. She was treated by antiangiogenic therapy (sunitinib). Under this treatment, she presented some large, extensive, severe and necrotizing ulcerations of both hands and feet, exacerbated with a sepsis. Sunitinib was stopped and antibiotics were combined with surgical trimming leading to clinical remission and complete healing. Sunitinib inhibits both tumor angiogenesis and tumor cell proliferation, but also the preexisting microcirculation. In our case, severe neuropathy caused neurovascular dysregulation which, together with hypertensive microangiopathy, led to a severe hand-foot skin reaction. This microangiopathy worsened under anti-VEGF therapy. The clinical severity was linked to the severity of the neuropathy. To avoid having serious cutaneous consequences, neuropathy and microangiopathy have to be diagnosed before introducing antiangiogenic therapy.

[Orofacial manifestations of systemic sclerosis: a study of 30 consecutive patients]. / Les manifestations buccofaciales de la sclérodermie systémique: étude de 30 patients consécutifs.

INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87%, mean age 58.6+/-13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33% of cases), or osteolytic lesions (7%) is poorly known.

[Risk of venous thromboembolism in acute medical illnesses. Part 2: Situations at risk in ambulatory, hospital and internal medicine settings]. / Risque thromboembolique veineux au cours des affections médicales aiguës. Partie 2: situations à risque en milieu ambulatoire, en milieu médical hospitalier et en médecine interne.

PURPOSE: The increased risk of thromboembolism in acute medical illnesses (AMI) is difficult to assess because of the diversity of medical conditions. The first part of this review of the literature was dedicated to methods of risk analysis based on our current pathophysiological knowledge. This second part describes more specifically the risk of venous thrombosis linked to AMI in hospital, ambulatory and internal medicine settings. CURRENT KNOWLEDGE AND KEY POINTS: The incidence of venous thromboembolism is higher in hospital than in ambulatory setting, albeit the latter remains significant. Stroke and affections leading to intensive care management represent conditions at great risk. Several mechanisms leading to a prothrombotic state have been identified, explaining the increased risk observed during relapses of pathologies specifically treated in internal medicine such as lupus erythematosus, Wegener granulomatosis, inflammatory bowel diseases and Behcet's disease. FUTURE PROSPECTS AND PROJECTS: Next to the pathophysiological understanding of venous thrombosis, the assessment of the specific thrombogenic burden of an AMI is an additive tool to screen medical patients at high risk. This systematic review of the literature shows uncertainties towards some risk factors as bedrest or acute inflammatory response. Taking into account the methodological difficulties inherent to prospective and epidemiological studies, a meta-analysis focusing on these factors would be useful to refine prevention guidelines for venous thromboembolism in medical setting.

[Risk of venous thromboembolism in the course of acute medical illness. Part 1: Basic research, clinical models, descriptive and analytic epidemiology]. / Risque thromboembolique veineux au cours des affections médicales aiguës. Partie 1: modèles pathogéniques fondamentaux et cliniques, épidémiologie descriptive et analytique.

PURPOSE: Some acute medical illnesses are associated with an increased risk of venous thromboembolism. This risk is difficult to assess in medical patients on account of the multiplicity of their pathologies, leading to potential interactions between numerous risk factors. CURRENT KNOWLEDGE AND KEY POINTS: A rational analysis of the risk of venous thrombosis in acute medical setting may be derived from pathogenic models and be based on elementary risk factors for venous thromboembolism classified as transient, permanent, major and intermediate factors. The systematic study of pathophysiological mechanisms in process, in each medical patient could guide the physician attitude toward still non-evaluated situations. FUTURE PROSPECTS AND PROJECTS: A synthesis of the last epidemiological and pathophysiological data is developed in the first part of this literature review, whereas the risk linked to specific situations in ambulatory, hospital or internal medicine settings will be described in the second part.

[Screening of abdominal aortic involvement using Doppler sonography in active giant cell (temporal) arteritis at the time of diagnosis. A prospective study of 30 patients]. / Dépistage de l'atteinte aortique abdominale par échographie-doppler au diagnostic de maladie de Horton. Etude prospective de 30 patients.

BACKGROUND: Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis. PATIENTS AND METHODS: This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta. RESULTS: Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm. CONCLUSION: Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

[Heredity and genetic aspects of Raynaud's disease]. / Aspects héréditaires et génétiques de la maladie de Raynaud.

The pathophysiology of primary Raynaud's phenomenon (Raynaud's disease) remains uncertain but the transmission of this primary microcirculatory dysregulation seems strongly influenced by genetic factors. For a long time, physicians have found that the hereditary factor plays an important role in the genesis of Raynaud's disease. Familial analysis and twin studies have confirmed the role of an hereditary factor. It seems heterogeneous but pedigree analysis indicates the possibility of an autosomal dominant transmission influenced by sex, in some families, allowing an approach called "reverse genetic" based on linkage analysis. Such an approach has focused on few loci but sequencing of candidate genes for genetic mutations remains negative. Given the supposed heterogeneity of the genetic transmission of Raynaud's disease, diversification of strategies in molecular genetics is suitable with reference to techniques applied to multifactorial heredity.

[Drug addiction and cardiovascular pathologies]. / Toxicomanie et pathologies cardiovasculaires.

Drug addiction which entails cardiovascular risks unknown or misknown to physicians, currently involves an increasing number of miscellaneous drugs, existing in manifold forms. There appears to be no bounds on the way of intake. All territories of the body may be affected with more or less severity. In young people, the cardiac, coronary, cerebral and peripheral vascular systems are generally involved. Two illicit drugs, cannabis and cocaine, showing a permanent increase in misuse, prevail. This drug addiction comes along with intercurrent pathologies which have their own vascular toxicity, especially HIV infection. Moreover, the advent of new illicit substances emphasizes the complexity of the clinical presentations. These complex situations have a real social and medical impact. We are currently in a phase of permanently increasing risk of cardiovascular complications. The pathophysiological mechanisms involved are intertwined and complicated by the frequent association of polytoxicomania or by the effects excipients added to these drugs: direct vascular toxicity, angeitis, arterial and venous thrombosis. Arsenic, a common component of these drugs, is also found in cigarettes; arsenic toxicity mainly affects the lower limbs. Treatment of these complications is non-specific; the ideal solution being weaning which, unfortunately in this peculiar population of patients, may entail serious complications due to the misuse of substitution products.