[Chronic severe uveitis: classification, search for etiology and therapeutic approach]. / Uvéites chroniques sévères : classification, démarche diagnostique et principes thérapeutiques.

PURPOSE: Severe chronic and refractory uveitis is a major diagnostic and therapeutic challenge for ophthalmologists and internists. Molecular tools, such as PCR but also new imaging techniques, have significantly changed the diagnostic approach during the last 10 years. Presumed and empirical diagnosis should be excluded in the face of atypical clinical presentations. CURRENT KNOWLEDGE AND KEY POINTS: A retrospective study based on 927 consecutive patients presenting with severe uveitis between 1991-1996, has recently defined the epidemiological characteristics and the visual outcome of this group of patients. An associated condition was determined in 67.5% of cases, divided in 4 different subgroups: infectious uveitis; uveitis associated with a systemic disease; eye-limited, presumed immune-mediated disorder and idiopathic eye-limited disorder. The management of patients with sight-threatening forms of uveitis is efficiently performed in collaboration with internists and depends on a complete diagnostic procedure and a well-adapted treatment. FUTURE PROSPECTS AND PROJECTS: Extensive work-up is mandatory when the therapeutic response seems atypical with resistance to corticosteroids and classical immunosuppressive drugs. Infectious uveitis should be excluded in severe and intractable forms of uveitis. Thereafter, new therapeutic strategies based on type I interferon and anti-TNF molecules can be proposed in order to decrease the potential risk of blindness in this young group of patients.

[Interferons and Behçet's disease]. / Interféron et maladie de Behçet.

BACKGROUND: Behçet's disease is a vasculitis of unknown origin. Various therapeutic agents have been proposed. Interferon alpha 2a or 2b appear to be the most promising. CURRENT KNOWLEDGE AND KEY POINTS: Numerous anecdotal reports and some short series have shown the efficacy of interferon in some cases previously resistant to conventional therapy, particularly to immunosuppressors. Posology, depending on the author, may vary from three million three times a week to 18 million three times a week. Interferon alpha is useful in cases of ocular, muco-cutaneous and articular involvement. Data is lacking for digestive, vascular and neurological manifestations. FUTURE PROSPECTS AND PROJECTS: Multicenter trials will nevertheless be required to determine the duration of therapy, risk of relapses, associated therapy and the optimal time to commence interferon treatment.

[Neurological manifestations of Behçet's disease]. / Manifestations neurologiques de la maladie de Behçet.

The neurological complications of Behçet's Disease are observed in 5 to 35 percent of cases depending of series. They included vascular and central nervous system manifestations. Vascular involvement is dominated by cerebral venous thrombosis marked by benign intracerebral hypertension. Prognosis is favourable with steroids and anticoagulation. Arterial involvement (thrombosis or aneurysm) are seldomly observed but increasingly with angio-MRI. Parenchymal involvement is dominated by meningoencephalitis eventually associated with fever. Myelitis and cranial nerve palsy are seldomly encountered. Cerebrospinal fluid is abnormal with lymphocytosis or pleiocytosis. MRI has a high sensitivity with hypersignals in the brainstem, basal ganglia, internal capsule and thalamus. Prognosis of central nervous system involvement is severe. Initially recovery is frequent, but severe impairment occurs due to relapse. In our experience, therapy including steroids, immunosuppressive drugs and colchicine improve short term and long term prognosis due to significant reduction of relapse in patients with good observance.

[Pregnancy and acute disseminated lupus erythematosus]. / Grossesse et lupus érythémateux aigu disséminé.

Until recently, pregnancy was thought to induce lupus flares; pregnancy was contraindicated and therapeutic abortion was recommended. Such concepts are now subjected to debate and successful outcome of pregnancy can be expected when lupus has not been active for several months, and with a successful partnership between specialists in high-risk obstetrics and in rheumatology or internal medicine.

[Aneurysm of the thoracic aorta and spondylodiscitis disclosing brucellosis]. / Anévrysme de l'aorte thoracique et spondylodiscite révélant une brucellose.

Brucellosis, which decreased during the eighties in France, remains a public health problem in many Mediterranean countries. We report the case of a 65-year old patient native of Morocco, of thoracic aorta aneurysm and lumbar spondylodiscitis due to Brucella melitensis, revealed by haemoptysis and lumbar pains, with a favourable outcome after aortic graft resection, spinal plaster immobilization and specific lengthy antibiotic treatment. This case report is characterized by the absence of endocarditis or infectious focus near the aneurysm. Diagnosis of aneurysm and spondilitis due to Brucella melitensis is based on imaging and bacteriological and serological examination. Because of a clinical and biological intolerance for rifampicin and cotrimoxazole, this patient received ofloxacin-doxycyclin-streptomycin. We discuss antibiotic recommendations and stress the interest of the early diagnosis of complicated forms of brucellosis for a better prognosis.

[Hydatid cyst of the interventricular septum. Contribution of magnetic resonance imaging]. / Kyste hydatique du septum interventriculaire. Apport de l'imagerie par résonance magnétique.

Cardiac localisation of hydatid cysts is rare representing 0.5 to 2% of all clinical forms of this condition. Hydatid cysts are usually observed in the left ventricle and involvement of the interventricular septum is exceptional. The principal diagnostic and preoperative investigations are serology and imaging by echocardiography and computed tomography, which can give conflicting results. The authors report a case of hydatid cyst of the interventricular septum in which computed tomography was non-contributory whereas echocardiography (the key investigation in all cases of hydatid cyst) associated with nuclear magnetic resonance imaging provided particularly accurate preoperative information. The latter investigation also allowed diagnosis of pulmonary, hepatic, splenic and renal involvement of the disease.