Fatal Systemic Vasculitis Associated with Chronic Active Epstein-Barr Virus Infection.

Chronic active Epstein-Barr virus infection is a rare disease with an often fatal outcome. Cardiovascular complications are associated with a poor prognosis. We herein describe the clinical course of an adult patient with Epstein-Barr virus-associated systemic vasculitis complicated by multi-systemic aneurysmal disease. The vascular imaging showed multiple aneurysms involving coronary arteries, abdominal arteries, cerebral arteries, and vertebral arteries. Immunophenotyping analysis of peripheral blood lymphocytes revealed the presence of an increased number of double negative T cells. The patient received multiple lines of immunosuppressive therapy with no response. Unfortunately, he succumbed to a cerebral aneurysm rupture.

Reactivation of cytomegalovirus in a patient with stevens-johnson syndrome-toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse cutaneous reactions to drugs. We describe the case of a 19 year old patient with SJS/TEN overlap syndrome, who developed severe interstitial pneumonia after she had received antiepileptic drugs. A cytomegalovirus infection was diagnosed by Real Time Polymerase Chain Reaction (RT-PCR) detection on Bronchoalveolar lavage. Based on observations on biological data, temporal relationship, and clinical features, it could be inferred that the reactivation of cytomegalovirus with viral replication can predispose a person to TEN-SJS. We discuss here, in the light of the current literature, the probable association between drug-induced SJS-TEN and fulminant reactivation of cytomegalovirus.

[Behcet syndrome: thirty comments with lung and vascular injury of peripheral vessels]. / Maladie de Behçet: trente observations avec atteintes vasculaires pulmonaires et des vaisseaux péripheriques.

INTRODUCTION: Behcet's disease is a systematic vasculitis of unknown cause, characterized essentially by eye, cutaneous, articular, neurological and vascular manifestations. METHODS: We retrospectively analysed the Behcet's disease cases that were followed up in our ward from January 2000 to January 2009. The inclusion criteria were those of International Study Group on Behçet's disease (aphthosis mouth was required). Data were retrieved and analysed with two softwares (Access(®) and Epi Info(®)). RESULTS: We observed 30 cases with vascular lesions on a series of 92 patients with Behcet's disease. Most patients were male, with an average age around 40. The venous manifestations, concerning essentially the lower limbs (deep and superficial thrombosis) were found at 27 patients (90 %), and the average of age during the appearance of the venous lesions was 40 years. Arterial lesions appear more late in 13 patients (43 %) (average of age 43 years). We noted, on the other hand, 11 cases of aneurysms and five cases of arterial thrombosis. The use of corticosteroids was necessary in all cases in association with the others drugs (anticoagulants, colchicine, immunosuppressors). Among the patients having had aneurysms, six were treated surgically. The outcome was favorable for most patients. Two patients had pulmonary embolism and two post-surgery complications. One patient died in the consequences of an intragastric break of an aneurysm of the abdominal aorta. CONCLUSION: The vascular involvement in Behcet's disease is manifested primarily by thrombophlebitis. Achieving blood pressure, less common, is problematic therapeutic because of the recurrent and life threatening.

[Antagonists of interleukin-6 (tocilizumab), in adult refractory still disease]. / Les antagonistes de l'interleukine-6 (tocilizumab) dans la maladie de still réfractaire de l'adulte.

INTRODUCTION: The pathogenesis of Still's disease is best elucidated for the better recognition of the involvement of Many pro-inflammatory cytokines in the genesis of this condition. Publications have reported the contribution beneficial for certain biotherapeutics, such as anti-TNFa, the anti-CD20 or antagonists of interleukine1 (IL-1) tested successfully in the treatment of systemic Juvenile idiopathic arthritis (Still's disease the child), the tocilizumab is a humanized monoclonal antibody directed against the receptor for interleukin-6 and is beginning to be reported as effective in some refractory cases of Still's disease in adults. PATIENTS: We report two young patients with Still's disease in adults with refractory early and prolonged remission after the first infusion tocilizumab. CONCLUSION: The tocilizumab can be used in patients MSA with refractory after failure or intolerance conventional treatments.