S. Hamzaoui

Pancréatite aiguë et syndrome hémophagocytaire au cours d'une poussée lupique : À propos d'une observation

Resume La pancreatite aigue et le syndrome hemophagocytaire (SH) sont rares au cours du lupus erythemateux systemique (LES). En revanche, l’association des 2 complications au cours d’une poussee lupique n’a jamais ete rapportee auparavant. Une jeune femme âgee de 31 ans, suivie pour LES depuis 7 ans a ete admise pour douleurs abdominales et fievre. Le diagnostic de pancreatite aigue a ete retenu devant une hyperamylasemie et l’augmentation du volume du pancreas avec collection intersplenorenale a la tomodensitometrie. Devant la pancytopenie, la cytolyse, la cholestase, l’hypertriglyceridemie et l’augmentation des lacticodeshydrogenases (LDH), un SH a ete suspecte et confirme par le myelogramme. L’evolution a ete favorable sous fortes doses de corticoides. Pres de 80 cas de pancreatites ont ete rapportes au cours du LES. L’etiologie est parfois difficile a etablir : le LES lui meme, les traitements et en particulier les corticoides, dont la responsabilite est controversee, et les infections favorisees par l’immunodepression. Quelques 40 cas de SH sont rapportes au cours du LES parfois en association avec une infection. La mortalite est de 38,7 %. Mais lorsque le SH constitue la manifestation initiale du LES ou survient au cours d’une poussee evolutive, l’evolution peut etre favorable sous corticoides.Exceptionally, acute pancreatitis and reactive hemophagocytic syndrome (RHS) are observed in the course of systemic lupus erythematosus (SLE). However, the association of the two conditions has never been reported before. A 31-years-old woman with a 7-year history of SLE was admitted for abdominal pain and fever. Elevated serum amylase and pancreatic enlargement on computerized tomography confirmed the diagnosis of pancreatitis. Laboratory examinations revealed pancytopenia, abnormal hepatic tests, and elevation of serum LDH and triglyceride levels. Bone marrow aspiration showed hemophagocytosis. The patient responded well to high dose corticosteroids. About eighty cases of pancreatitis have been reported in patients with SLE. The mechanisms are still unclear: SLE as the primary etiologic factor, drug toxicity, especially steroids which play a controversial role, or infection. About 40 cases of RHS have been reported in patients with SLE, sometimes associated with active infection. Overall mortality is 38.5%. When RHS occurs as an initial manifestation of SLE, or in the course of active SLE, it responds well to immunosuppressive therapy.

Profil étiologique des nécroses digitales des membres supérieurs : analyse de 25 observations

AIM To investigate the etiologies of the upper limb digital necrosis based on a retrospective analysis of 25 cases. PATIENTS AND METHODS We retrospectively reviewed the medical records of patients treated for digital necrosis of the upper limb in four departments of internal medicine from January 1997 to December 2003. RESULTS There were 16 women and nine men, mean age 55 years. Eleven patients were smokers. Raynauds phenomenon was noted in 12 cases. Connective tissue diseases were the most common cause (nine cases), all of them were women. The second cause was atherosclerosis (five cases) and Buergers disease (five cases). In the other cases, the following diagnoses were found: vasculitis (three cases) and neoplasm (two cases). No cause could be identified in one female smoker. CONCLUSION Digital necrosis is a common symptom, revealing a vascular pathology. Its causes are diverse. In women, it first suggests a connective tissue disease whereas in men, a diffuse arteriopathy. The etiological diagnosis strategy should consider drug intake, anamnesis and Raynauds phenomenon history. However, in all cases the etiology investigations should not delay the treatment in order to preserve functional prognosis.

Troubles de la perfusion hépatique secondaires à une thrombose cave supérieure : à propos de trois cas

Resume Le but de ce travail est d’evaluer les alterations de la perfusion hepatique qui peuvent etre observees en cas de thrombose de la veine cave superieure. A travers l’exposition de trois cas de thrombose de la veine cave superieure, les auteurs expliquent les differentes collateralites mises en jeu creant des anastomoses systemo-portales a l’origine de ces troubles de la perfusion ainsi que leur traduction en tomodensitometrie.

Vascularite urticarienne hypocomplémentémique de McDuffie associée à un syndrome de Gougerot Sjögren

We report a patient with Sjögrens syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmers test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids.

Imaging Features of Behçet’s Disease

Behcet’s disease is a chronic vasculitis clinically characterized by relapsing orogenital aphthosis associated with a large multisystemic spectrum of clinical manifestations. Imaging is essentially helpful to evaluate vascular involvement and to monitor post-therapeutic evolution of cardiovascular, neurological, respiratory, and digestive involvements. Familiarity with the various and specific imaging findings of this disease is fundamental to make an early detection and guide the optimal care of patients.