M. Badaoui

Les antagonistes de l’interleukine-6 (tocilizumab) dans la maladie de still réfractaire de l’adulte

INTRODUCTION The pathogenesis of Stills 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 (Stills 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 Stills disease in adults. PATIENTS We report two young patients with Stills 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.

Un abcès rétropharyngé

Une femme de 16 ans, sans antécédents pathologiques, était dmise pour une exploration d’une tuméfaction latérocervicale roite, avec dysphagie haute, évoluant depuis un mois et assoiée à une fébricule et un amaigrissement non chiffré. L’examen de a région cervicale retrouvait une adénopathie jugulocarotidienne aute droite, douloureuse, inflammatoire, de consistance molle et esurant 3 cm de grand axe. L’examen endobuccal mettait en évience un bombement de la paroi postérieure du pharynx, ainsi que eux adénopathies sous-maxillaires gauches. Le reste de l’examen tait sans particularité. Le scanner cervical (Fig. 1) montrait une collection de densité iquidienne, mesurant 25 × 18 × 50 mm, de siège rétropharyngé, ’étendant en haut jusqu’au plancher du cavum qui était refoulé en aut et arrivant en bas au niveau du C3. L’adénopathie jugulocarotiienne sus-décrite paraissait à centre nécrotique. Sur l’IRM (Fig. 2), ette collection ovalaire et bien limitée était en hyposignal T1, en ypersignal T2 et en contact en arrière avec la carotide primitive roite, et rétrécissant la lumière du rhinopharynx. La radiographie es poumons et le scanner thoraco-abominopelvien étaient sans nomalies. Il n’avait pas d’anémie, ni de syndrome inflammatoire iologique ni de cytolyse hépatique.

RACAND syndrome associated with primary biliary cirrhosis

The acronym RACAND means the association of Raynauds phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynauds phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells.

Cas cliniqueSyndrome RACAND associé à une cirrhose biliaire primitiveRACAND syndrome associated with primary biliary cirrhosis

The acronym RACAND means the association of Raynauds phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynauds phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells.

Syndrome RACAND associé à une cirrhose biliaire primitive

The acronym RACAND means the association of Raynauds phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynauds phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells.