Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome.

Primary adrenal lymphoma is rare. It is often bilateral and in most of the cases of B-cell type. The clinical features are various and not specific. We report a case of a 69-year-old woman who had a diffuse large B-cell lymphoma associated with hemophagocytic syndrome. The abdominal imaging reveals the existence of bilateral adrenal hypertrophy. A CT scan-guided biopsy concluded to a diffuse large B-cell lymphoma CD 20-positive associated EBV. The treatment consisted on "CHOP like" chemotherapy associated with rituximab. Primary adrenal lymphoma has a poor prognosis, even more poorly if associated with hemophagocytic syndrome.

[Lupus hepatitis: a case series of 12 patients]. / Hépatite lupique: une série de 12 patients.

PURPOSE: Several liver manifestations have been reported in systemic lupus erythematosus (SLE) and are usually non specific. We report on our experience of lupus hepatitis. METHODS: A retrospective monocenter study of 73 patients with SLE. The diagnosis of lupus hepatitis was established after exclusion of other causes of hepatitis and hepatic vein thrombosis. RESULTS: Liver involvement was noted in 12 patients (16.4%). There were nine female and three male patients; the mean age of these patients was 29 years. In seven patients liver involvement was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the five remaining patients. In all patients, liver manifestations were associated with other organ involvement. Clinical manifestations were: hepatomegaly (n=4), jaundice (n=4), abdominal pain (n=3), ascites (n=2), portal hypertension (n=1) and hepatic failure with encephalopathy (n=1). Elevated liver enzyme was noted in 11 cases and liver cholestasis in eight cases. Presence of anti-ribosomal P antibodies was noted in one case. Liver biopsy was performed in five patients, and revealed chronic active hepatitis in three cases, chronic hepatic granulomas in one case and nonspecific inflammation in one case. The outcome was favorable in 11 patients without relapse, and one patient died of encephalopathy and liver failure. CONCLUSION: Liver involvement associated with SLE is not uncommon. It is frequently asymptomatic and limited to liver test abnormalities. The role of anti-ribosomal P autoantibodies remains uncertain.

[Chronic intestinal pseudo-obstruction in systemic lupus erythematosus]. / Pseudo-obstruction intestinale au cours du lupus érythémateux systémique.

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.

[Takayasu's disease in central Tunisia: 27 cases]. / La maladie de Takayasu dans la région centre de la Tunisie. A propos de 27 cas.

BACKGROUND: Takayasu's arteritis is a rare inflammatory disease and few data are available in Tunisia. The aim of this study is to evaluate clinical and radiological features of the disease in the centre of Tunisia. METHODS: We retrospectively studied medical records of patients treated in departments of internal medicine or cardiology from three university hospitals in Sousse and Monastir over the period 1985-2005. The criteria for inclusion were those proposed by the American College of Rheumatology. RESULTS: Twenty-seven patients were identified. The mean age at presentation was 33.2 years (range 16-68 years) and 88.9% were female. The mean delay from the onset of the symptoms to the time of diagnosis was 4.2 years. Intermittent claudication was the most common presentation (81.5%) and hypertension was noted in 40.7% of cases. Arterial localization most frequently involved was subclavian artery. The aorta was involved in 52.3% and renal arteries in 36.3% of cases. Stenosis or occlusions was constant but aneurysms were noted in 7.4%. Functional difficulty was the main complaint in the follow-up, death related to Takayasu's disease was noted in 3.7%. The mean follow-up time was 75.8 months (6.3 years). CONCLUSION: There is no epidemiologic particularity of Takayasu's disease in Tunisia, however involvement of the subclavian artery was more frequent than the aortic localization.