QUAND RECHERCHER UNE HYPERTENSION ARTERIELLE PULMONAIRE AU COURS D’UNE MALADIE SYSTEMIQUE

Introduction: Pulmonary hypertension is a rare but well-known life-threatening complication of connective tissue diseases. The aim of this article is to analyse the available literature for diagnosis and treatment about this complication. Method: Scleroderma is the most common connective tissue disease affected by pulmonary hypertension. Dyspnea is the main symptom and is frequently severe. Echocardiography is an excellent exam to detect pulmonary hypertension. However, right heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide. Pulmonary hypertension is less severe in patients with connective tissue diseases perhaps because of an earlier diagnosis. A significantly lower proportion of patients present an acute vasodilator response, suggesting an early constitution of irreversible pulmonary vascular lesions. Continuous intravenous epoprostenol therapy seems to be less effective as compared with patients with primitive pulmonary hypertension and does not improve survival. Bosentan and Sildenafil are approved in idiopathique pulmonary hypertension. The role of immunosuppressive therapy in lupus erythematous has been reported. Conclusion: The WHO recommended annual detection of scleroderma pulmonary hypertension with echocardiography in asymptomatic patients. However, half of patients have dyspnea III or IV NYHA at the diagnosis. The prognostic is more severe than idiopathic pulmonary hypertension.Introduction : L’hypertension artérielle pulmonaire est une complication rare mais bien connue des connectivites. L’objet de cet article est de faire le point sur les méthodes diagnostiques et les nouveautés thérapeutiques Méthodes : La sclérodermie est la première connectivite à se compliquer d’hypertension artérielle pulmonaire. La dyspnée d’effort est le maître symptôme et est souvent d’emblée sévère. L’échocardiographie est un excellent examen de dépistage mais le cathétérisme cardiaque droit demeure l’examen de référence indispensable pour confirmer le diagnostic. Le traitement vasodilatateur par perfusion continue de prostacycline n’améliore pas la survie de ces patients contrairement à ce que l’on observe dans l’hypertension artérielle pulmonaire primitive. Le bosentan et le sildenafil ont approuvé leurs efficacités au cours de l’hypertention artérielle pulmonaires idiopathiques. L’HTAP du lupus érythémateux systémique peut être sensible à l’Endoxan et aux corticoïdes. Conclusion : Les recommandations de l’OMS sont de dépister annuellement l’HTAP chez tout patient atteint de sclérodermie systémique même en l’absence de symptôme clinique par échocardiographie. Malgré ces recommandations, plus de 50 % des patients développant une HTAP sur sclérodermie sont en classe III ou IV NYHA au moment du diagnostic. Le pronostic reste plus sombre que celui de l’HTAP idiopathique

SYNDROME DES ANTISYNTHETASES

Introduction: The discovery in recent years, specific antibodies of idiopathic inflammatory myopathies, has opened new avenues in the understanding of their physiopathological mechanisms.We present the main clinical features and prognosis of syndromes associated with antibodies antisynthetase groupe, emphasizing the anti-Jo1.Method: The antisynthetase syndrome is a subgroup of idiopathic inflammatory myopathies, characterized by interstitial lung disease, arthritis, Raynaud's phenomenon and cutaneous "mechanics hands" associated with a specific antibody family of antisynthetases, anti-Jo1 and pulmonary life-threatening conditions. Treatment is not codified; corticosteroids are most effective on the fickle lung injury by discussing other immunosuppressive treatments.Conclusion: The antisynthetase syndrome is an entity to be known, because of the severity of interstitial lung disease, may occur in the absence of myopathy. Most authors recommend looking for the anti-Jo1 in any idiopathic interstitial pneumonia.Introduction : La découverte ces dernières années, d’anticorps spécifiques des myopathies inflammatoires idiopathiques, a ouvert de nouvelles voies dans la compréhension de leurs mécanismes physiopathogéniques. Nous présentons les principales caractéristiques cliniques et pronostiques des syndromes associés à la famille des anticorps antisynthétases, en insistant sur l’anticorps anti-Jo1.Méthode : Le syndrome des anti-synthétases constitue un sous-groupe de myopathies inflammatoires idiopathiques, caractérisé par une atteinte interstitielle pulmonaire, une polyarthrite, un phénomène de Raynaud et une atteinte cutanée de type « mains de mécaniciens », associés à un anticorps spécifique de la famille des anticorps anti-synthétases, l’anti-Jo1. L’atteinte pulmonaire conditionne le pronostic vital. Le traitement n’est pas codifié ; les corticostéroïdes ont une efficacité inconstante sur les lésions pulmonaires, faisant discuter d’autres traitements immunosuppresseurs.Conclusion : Le syndrome des antisynthétases est une entité à connaître, du faite de la gravité de la pneumopathie interstitielle, pouvant survenir en l’absence d’atteinte musculaire. La plupart des auteurs recommendes la recherche de l’anticorps anti-jo1 lors de toute pneumopathie interstitielle idiopathique

Predictive model of biliocystic communication in liver hydatid cysts using classification and regression tree analysis

<p>Abstract</p> <p>Background</p> <p>Incidence of liver hydatid cyst (LHC) rupture ranged 15%-40% of all cases and most of them concern the bile duct tree. Patients with biliocystic communication (BCC) had specific clinic and therapeutic aspect. The purpose of this study was to determine witch patients with LHC may develop BCC using classification and regression tree (CART) analysis</p> <p>Methods</p> <p>A retrospective study of 672 patients with liver hydatid cyst treated at the surgery department "A" at Ibn Sina University Hospital, Rabat Morocco. Four-teen risk factors for BCC occurrence were entered into CART analysis to build an algorithm that can predict at the best way the occurrence of BCC.</p> <p>Results</p> <p><b>I</b>ncidence of BCC was 24.5%. Subgroups with high risk were patients with jaundice and thick pericyst risk at 73.2% and patients with thick pericyst, with no jaundice 36.5 years and younger with no past history of LHC risk at 40.5%. Our developed CART model has sensitivity at 39.6%, specificity at 93.3%, positive predictive value at 65.6%, a negative predictive value at 82.6% and accuracy of good classification at 80.1%. Discriminating ability of the model was good 82%.</p> <p>Conclusion</p> <p>we developed a simple classification tool to identify LHC patients with high risk BCC during a routine clinic visit (only on clinical history and examination followed by an ultrasonography). Predictive factors were based on pericyst aspect, jaundice, age, past history of liver hydatidosis and morphological Gharbi cyst aspect. We think that this classification can be useful with efficacy to direct patients at appropriated medical struct's.</p