Phénotypes cliniques et pronostic du syndrome des antisynthétases

Antisynthetase syndrome (ASS) was first described in 1989 as an inflammatory myopathy associated with the presence of specific auto-antibodies, namely the anti-tRNA-synthetase antibodies (ASA). To date, the ASA family comprises eight different auto-antibodies, among which anti-hystidyl-tRNA-synthetase (anti-Jo1) is the most prevalent. In addition to myositis, a constellation of clinical features has also been described in ASS, including interstitial lung disease, Raynaud's phenomenon, polyarthritis, fever and mechanic's hands. Large variations in the distribution and the severity of each of these symptoms are reported from one patient to another, and also over the course of the disease. The heterogeneity of this autoimmune connective tissue disease has led to difficulties in the early identification of patients with a poor outcome (those who will require the most intensive treatments). Additionally, very few prospective trials have so far compared the efficacy of the different immunosuppressive drugs available, and evidence is lacking to help adapting therapeutic strategies to all of the different ASS clinical situations. We will review the different characteristics of ASS (namely biological, clinical, functional, and morphological ASS parameters) that have recently been shown to correlate with patients’ outcome, our aim being to discuss the usefulness of patient stratification for elaborating targeted therapeutic trials for ASS in the future.     

Manifestations cliniques des infections à Chlamydia pneumoniae

Chlamydia pneumoniae is a newly described and ubiquitous bacterium. Most infections are asymptomatic as shown by a high worldwide séroprévalence (> 50% of cases). It is a common cause of acute respiratory infections, mainly pneumonia (> 50% of cases) and other acute respiratory tract infections (25% of acute bronchitis, < 5% of sinusitis, otitis and pharyngitis). About 10% of the community acquired pneumonia cases have been associated with Chlamydia pneumoniae infection. This incidence depends on a cyclic epidemiology with a high incidence for 2 to 3 years-followed by a low prevalence for 3 to 4 years. Most chlamydial infections are mild but occasionnaly severe with death especially in old people. Mostly acute infections are reccurent infections. The seroprevalence is higher in asthmatic patients, its role in acute exacerbation of chronic bronchitis is not definitely established. Extra-respiratory acute infections are less frequent, either fever alone, or cardiovascular diseases (acute myocarditis, pericarditis and endocarditis) or neurological (encephalitis, meningitis or Gudlain-Barré syndrome). In addition, seroepidemiology studies have shown an association with coronary artery disease, Chlamydia pneumoniae was detected in coronary atheroma by immunochemistry, polymerase chain reaction and by electron microscopy. Chlamydia pneumoniae may be involved in the atherosclerotc process. To define the clinical spectrum of infection requires precise laboratory diagnosis, the most efficient tests (PCR, direct immunofluorescence and culture) are done in specialized laboratories, serological tests are less reliable. Macrolides, cyclines and fluoroquinolones are the most potent antibiotics but with differences in vitro within and between these families of antibiotics. Bacteriological failures are described despite the in vitro activity. A lot of questions on clinical aspects, epidemiology and treatement are unanswered, we need more studies.     

Déni de grossesse et néonaticide : aspects cliniques et psychopathologiques

Pregnancy denial and neonaticide have recently received media coverage following a series of French cases of neonatal killing. Although it has been known for a long time that some women deny their pregnancy and may kill their newborns, there is still no consensus on the etiopathogenic factors involved in the denial of pregnancy occurrence. Even though neonaticide is often committed by young, poor, unmarried women with little or no prenatal care, it appears that denial of pregnancy is a heterogeneous condition associated with different psychological features. Societies are ambivalent with regard to mothers who killed their children and tend to lay the entire blame on them. Furthermore, there is a widespread lack of understanding among the public on these affairs, when birth control techniques and methods are widely available. The purpose of this article is to describe the different types of pregnancy denial and neonaticide and to review the still debated etiopathogenic hypotheses. The absence of the physical changes of pregnancy at the time of the denial such as cessation of menstruation, abdominal swelling or perception of foetal movements is also discussed.     

IRM cardiaque : technologie actuelle,applications cliniques et perspectives futures

The field of cardiovascular MRI has evolved rapidly over the past decade, feeding new applications across a broad spectrum of clinical and research areas. Advances in magnet hardware technology, and key developments such as segmented k-space acquisitions, advanced motion encoding techniques, ultra-rapid perfusion imaging and delayed myocardial enhancement imaging have all contributed to a revolution in how patients with ischemic and non-ischemic heart disease are diagnosed and treated. Actually, cardiac MRI is a widely accepted method as the “gold standard” for detection and characterization of many forms of cardiac diseases. The aim of this review is to present an overview of cardiac MRI technology, advances in clinical applications, and future directions.     

Aspects cliniques des allergies cutanées graves (toxidermies exclues)

Some skin diseases require emergency medical intervention when they are life-threatening or when the eruption is spectacular and brutal and the patient has an elevated temperature. We will discuss the clinical presentations of these conditions, excluding cutaneous drugs eruptions. Patients with severe atopic dermatitis may require brief hospitalisation if their eczema is generalized, refractory to classical therapy, or there are infectious complications, as in the Kaposi-Juliusberg syndrome when the condition is associated with a secondary herpetic infection. Acute urticaria and angioedema are frequent causes of emergency room-visits. They can be the result of drug allergy, food allergy, allergy to stinging insects or contact urticaria. However, the cause is not always identified, even after a complete allergy work-up. Some cases of contact eczema are spectacular, in particular when the face is severely oedematous. Paraphenylenediamine, topical corticosteroids and topical non-steroidal anti-inflammatory drugs are often the cause of severe delayed-type hypersensitivity reactions.