Y. Sekkach

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.

Atteinte oculaire au cours de la périartérite noueuse. Deux observations

INTRODUCTION Polyarteritis nodosa (PAN) is a systemic vasculitis with polymorphic manifestations. Ocular involvement is rare; we report two such cases. CASES The first case was a 56-year-old man with PAN treated with corticosteroids. A year and a half later, during a disease outbreak associated with decreased visual acuity, ocular examination objectified a localized pigment epithelial detachment in the right eye. The outcome was favorable with corticoid bolus followed by azathioprine. The second case was a 41-year-old man presenting with PAN and decreased visual acuity. Ophthalmologic examination showed inferotemporal branch vein occlusion in the right eye with serous retinal detachments in the left eye. The outcome was favorable with intravenous cyclophosphamide followed by azathioprine. DISCUSSION The incidence of ocular involvement in PAN varies from 10 to 20%. It is most often associated with vasculitis of retinal and choroidal arteries. Fluorescein angiography is the gold standard of diagnosis. Multifocal acute ischemia of the choriocapillaris is very pathognomonic. Immunosuppressant drugs are effective and should be reserved for severe forms.

Une tuméfaction douloureuse de la cuisse

Un homme, âgé de 23 ans, était hospitalisé pour une tuméfacion douloureuse de la cuisse droite. En dehors de deux épisodes écents d’aphtose buccale, il n’avait pas d’autres antécédents. Il vait consulté trois semaines auparavant pour une douleur isoée, non fébrile, de la face interne de la cuisse droite, d’installation rogressive et apparue sans facteur déclenchant. Malgré les traiteents antalgiques, les douleurs devenaient majeures en 48 heures. l’examen clinique, on notait une tuméfaction de la cuisse droite ensible, pulsatile et soufflante à l’auscultation avec une adénoathie inguinale homolatérale. Il y avait une hyperleucocytose à 2 000 par millimètre cube à prédominance de polynucléaires neurophiles, la vitesse de sédimentation était à 50 mm à la première eure, la CRP à 40 mg/l. Il n’y avait pas d’autre anomalie biologique. n angioscanner corps entier était pratiqué (Fig. 1 et 2).

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.

À propos d’une hyperparathyroïdie maligne historique à localisations métastatiques inhabituelles

We report a historical case of hyperparathyroidism in a young patient hospitalized for an array of osteolytic foci and incomplete fracture associated with a swollen neck, revealing a very special form of a metastatic parathyroid carcinoma with unusual multiple locations and exceptional medullary flooding. Carcinoma of the parathyroid gland produces a malignant hypersecreting tumor particularly difficult to diagnose. Treatment of this rare tumor is primarily surgical. The preoperative syndrome is unusually severe primary hyperparathyroidism. Intraoperatively, the size of the tumor and its local extension to surrounding tissue are highly suggestive. Confirmation requires pathological analysis of the operative specimens and can be further supported by the clinical course of local recurrence or metastasic spread. Specific immunohistochemical techniques have recently been shown to be contributive. The diagnosis is strengthened in the presence of associated Schantz and Castelman criteria. Foci of local extension can be identified preoperatively with ultrasound, (99m)Tc-sestamibi scintigraphy and MRI of the neck and mediastinum. The prognosis depends mainly on the possibility of achieving complete resection at the initial surgery. In some cases, very aggressive complementary postoperative radiotherapy is likely to improve locoregional control of the tumor. Chemotherapy alone or in combination with radiation has not demonstrated its effectiveness. The disease course and control can be monitored by regular assay of serum calcium and the parathormone.

Serum vitamin D and vitamin D receptor gene polymorphism in Moroccan patients with systemic lupus erythematosus

Background: Vitamin D plays an important role in the immunomodulation and could be involved in the development of autoimmune diseases such as systemic lupus erythematous (SLE). The study of the polymorphism of the Vitamin D Receptor (VDR) gene may be of interest in explaining the pathophysiology of SLE. Methods: In this study, we aimed to examine the characteristics of VDR gene BsmI polymorphism for the first time in Moroccan patients with SLE and their relationship with clinical manifestations of the disease. We also measured the serum level of 25-hyroxyvitamin D 3 to assess its relation to such polymorphism. Results: The study included 66 SLE patients and 91 healthy controls. Our results showed that there were no differences observed in VDR genotypes and allelic distribution within the two groups. Both groups were in Hardy-Weinberg equilibrium, with no significant P values for the observed and expected genotype frequencies. 25-hyroxyvitamin D3 serum levels were the same in the two groups. Conclusions: Based on the results of the present study. We cannot verify any association between VDR gene BsmI polymorphism and SLE. This polymorphism could not be regarded as a genetic marker of the SLE. A larger study examining BsmI and other VDR gene polymorphisms is needed.

Tétraparésie révélant un adénome de Conn chez une femme enceinte

We report the case of Conn adenoma revealed by tetraparesis in a 33-year old pregnant woman at the 16thweek of amenorrhea. The patient had a blood pressure of 147/87 mmHg, which was considered high-normal, hypokalemia at 1.1 mmol/l. The diagnosis was confirmed by hormone dosage which showed elevated plasma aldosterone levels and decreased plasma renin activity. MRI showed a left adrenal nodule, 1.5 cm in diameter, compatible with adrenal adenoma. Left adrenalectomy was performed with simple postoperative course and normalization of kalemia and blood pressure.

Des lésions bulleuses

Un homme, âgé de 34 ans, sans antécédent particulier, qui avait epuis six mois des arthralgies et un phénomène de Raynaud, tait hospitalisé pour exacerbation des symptômes avec appariion de lésions bulleuses depuis quatre semaines. L’examen clinique etrouvait un érythème malaire, des ulcérations buccales et des ulles intéressant le cou, le tronc et les membres supérieurs (Fig. 1). l n’avait pas d’érythème polymorphe et le signe de Nikolsky était égatif. Il était asthénique et avait perdu 12 kg. Les examens bioogiques montraient une leuconeutropénie (leucocytes : 3800 par illimètre cube, polynucléaires neutrophiles : 2000 par millimètre ube), une vitesse de sédimentation à 79 mm à la première heure. Le osage des anticorps antinucléaires, anti-DNA natifs, anti-SSA, antiSB étaient positifs, le latex à 320 UI/l et le Waaler Rose à 160 UI/l. es anticorps anti-Sm, anti-RNP, anti-Jo1, anti-C1q, anti-CCP et les nca étaient négatifs. Le dosage de la fraction C4 du complément tait effondré. La fonction rénale était conservée avec une clairance e la créatinine à 98 ml/min sans protéinurie.

Rhabdomyolyse sévère révélant une myopathie hypothyroïdienne d’origine auto-immune

While muscular manifestations are common of hypothyroidism, hypothyroid myopathy is most often limited to myalgia, muscular stiffness and cramps with, in some patients, elevated levels of muscle enzymes. We report two cases of rhabdomyolysis related to hypothyroid myopathy. One of the patients developed acute renal failure. Thyroid hormone replacement therapy improved thyroid and renal function with involution of rhabdomyolysis. Hypothyroidism appears to be an authentic cause of rhabdomyolysis and should be carefully ruled out in all patients with elevated serum levels of muscle enzymes.

Rhabdomyolyse sévère révélant une myopathie hypothyroïdienne d'origine auto-immune À propos de deux cas

While muscular manifestations are common of hypothyroidism, hypothyroid myopathy is most often limited to myalgia, muscular stiffness and cramps with, in some patients, elevated levels of muscle enzymes. We report two cases of rhabdomyolysis related to hypothyroid myopathy. One of the patients developed acute renal failure. Thyroid hormone replacement therapy improved thyroid and renal function with involution of rhabdomyolysis. Hypothyroidism appears to be an authentic cause of rhabdomyolysis and should be carefully ruled out in all patients with elevated serum levels of muscle enzymes.