Presse Med
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The inflammatory myopathies are a group of quite proteiform, systemic auto-immune diseases which include polymyositis, dermatomyositis and inclusion body myopathies. To facilitate the diagnosis, classification criteria (Bohan and Peter, 1975) have been proposed, based essentially on clinical criteria. In addition, over the past fifteen years, auto-antibodies characterizing certain forms of inflammatory myopathy have been identified. ⋯ The latter are at the moment very heterogeneous as they use techniques and above all antigenic preparations which are extremely diverse. These antibodies are also very interesting "physiopathological" tools to try to better understand myositis. The example of anti-tRNA synthetases is a particularly original model of auto-immunization, which allows one to establish a link between an initial, probably poorly specific muscular lesion and the appearance of auto-antibodies which maintain and aggravate the muscular disease.
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Medical problems during flight have become an important issue as the number of passengers and of miles flown continue to rise. Cabin pressurization causes hypoxia, hypobaria and decreased humidity, which are responsible for most medical incidents occurring during flight. Worldwide daily medical incidents are estimated at 350, i.e., one per 14,000 to 39,600 passengers. ⋯ Passenger flights carry medical equipment and drugs, determined according to number of passengers and the flight distance. The conditions of intervention are codified: "good Samaritan" laws protect professionals from liability when they choose to aid others who are injured or ill. Current recommendations call for physicians to identify themselves, request an interpreter when necessary, obtain the patient's consent, conduct out examination, inform the patient, family members and crew members of the situation, contact ground medical staff, use well-known procedures, consider flight diversion, and write up a case report.
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Treatments of systemic necrotizing vasculitides have progressed markedly over the past few decades. The first attempts to obtain better-adapted therapeutic strategies evaluated the indications of conventional drugs, and their abilities to prolong survival and prevent relapses, while decreasing the severity and number of side effects. ⋯ Recent therapeutic strategies include immunomodulating methods, like plasma exchanges, or products, like intravenous immunoglobulins, or, more recently, new agents called biotherapies. Some of the latter have achieved promising effects, for example, anti-tumor necrosis factor-alpha and anti-CD20 monoclonal antibodies, and are now being evaluated in prospective trials.
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Transthoracic echocardiogram is the best tool for the screening of PH. When PH is suspected, the diagnosis must be confirmed by a right heart catheterization, and a vasoreactivity testing with NO must be performed in all cases of pulmonary arterial hypertension. ⋯ Routine screening is warranted in systemic sclerosis, HIV infection and portal hypertension. All patients with PH must be referred to a reference or a competence center for PH.