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Pulmonary artery aneurysm in Behcet disease compromises seriously the diagnosis since its fatal rupture is still the major cause of death in this disease. Pulmonary artery aneurysm requires urgent management and several treatments have been proposed, including steroids, immunosuppressive drugs, embolisation and surgery. Herein, we report a patient with Behçet's disease in whom multiple pulmonary artery aneurysms were completely resolved after a combined treatment with corticosteroids and azathioprine. Pulmonary artery aneurysms in Behçet's disease are reported to indicate poor prognosis and high mortality. Computed tomographic scans of the chest and angiography are the most common diagnostic procedures used in the diagnosis or evaluation of pulmonary artery aneurysm. Immunosuppression is the main therapy for the treatment of a vasculitis.

Insulinoma is a rare tumor. Its annual incidence varies from 1 to 4 cases per million in the United States [1–3] but has not been determined in the different populations. We conducted a retrospective study to evaluate the incidence, the epidemiological characteristics, and the prognosis of insulinoma in Tunisia. Our study is the first to determine the annual incidence of insulinoma in Tunisia. It concerned all cases of insulinomas diagnosed in Tunisia between April 1980 and April 2009. Search was carried out in all university centers including endocrinology, internal medicine, pediatric, and surgery departments in Tunis (6 centers), Sfax, Sousse, Monastir, and Mahdia. We analyzed epidemiological data and patient’s medical records. Over the 29 years period, 21 patients with confirmed insulinoma were identified. So, the annual incidence of insulinoma in Tunisia was 0.1 case per million. This low incidence may be an underestimate because of the difficulties to find out all the cases of insulinoma. The patients were 10 females (47.6%) and 11 males (52.3%). Mean age at diagnosis was 46 years [8–80]. There was only one child in the series. Sex ratio and age at diagnosis were close to that described in other studies [1–5]. Mean diagnosis delay was 5 ± 6.9 years ranging from 4 months to 30 years. The initial presentation was neurogenic symptoms in 13 cases (62%), among them five (24%) with recurrent seizure. Insulinoma was diagnosed in a 49 years old woman with type 2 diabetes known for 14 years. Multiple endocrine neoplasia type I was not reported in our cases. Twenty patients were operated and follow-up data were available in 19 cases. The tumors were located at the head in 4 cases, at the body in 7 cases, and at the tail in 8 cases. The mean diameter was 25 mm, ranging from 3 to 115 mm. Eleven tumors measured 2 cm or more (52%). Insulinoma was multiple in one case (5%). Malignancy was present in three cases (18.7%). The operative mortality was 5.2% (one death secondary to biliary leakage). The recovery rate was 68.4% (13/19). Persistent hypoglycemia was reported in two cases (10.5%) among which one malignant insulinoma with metastasis. Recurrence was noted in three cases. With the results of this study, we can conclude that the incidence of insulinoma seems to be low in Tunisia. Due to the variability and the lack of specificity of clinical presentation, diagnosis of insulinoma was frequently delayed as indicated by the large size of the tumors. Malignancy was frequent. The cure rate was unsatisfactory. M. Chihaoui (&) R. Hmaissi H. Slimane Department of Endocrinology, La Rabta Hospital, Tunis, Tunisia e-mail: mm.chihaoui@planet.tn

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n=6), vomiting (n=3), and fever (n=3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.

Amyloidosis limited to bronchopulmonary apparatus is expressed as tracheobronchial deposits in diffuse plaques that can cause stenosis or parenchymal nodules or masses. In this regard, we report four cases of pulmonary amyloidosis and discuss the diagnostic difficulties of this location. These are two women and two men aged 60, 68, 44 and 57 years. They presented a pulmonary parenchymal amyloidosis in all cases associated with bronchial in one case. The diagnosis was confirmed by histology in all cases. The staging was negative in all cases. The evolution was marked by the stabilization of the lesions in all cases. Localized amyloidosis, which may be the only telltale sign of a systemic illness, its diagnosis requires finding other locations to better tailor the treatment strategy.

To investigate the etiologies of the upper limb digital necrosis based on a retrospective analysis of 25 cases.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.There were 16 women and nine men, mean age 55 years. Eleven patients were smokers. Raynaud's 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 Buerger's 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.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 Raynaud's phenomenon history. However, in all cases the etiology investigations should not delay the treatment in order to preserve functional prognosis.

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved.

Sarcoidosis is a systemic disorder of unknown aetiology that may involve many organs including the liver.This is a monocentre retrospective and descriptive study over 12 years of patients with systemic sarcoidosis who presented with liver involvement.Amongst 21 patients with systemic sarcoidosis, seven presented a liver involvement (five males and two females; mean age 42.5 years). The liver involvement was the presenting manifestation in three. Liver enlargement was noted in five patients and abnormal liver function tests in four. In one patient, abdominal computed tomographic scan showed a nodular hepatomegaly. Liver histology demonstrated typical granulomatous lesions in six. One patient developed a Budd-Chiari syndrome that responded favourably to corticosteroids. Six out of the seven patients were treated with corticosteroids.Liver involvement in common in sarcoidosis and commonly asymptomatic. Treatment is not systematic.

Islet-cell tumors are the most common neuroendocrine tumors that arise from the endocrine pancreas. They are typically benign and sporadic. Diagnosis is generally established late because clinical signs lack specificity. The insulinoma is difficult to localize since it is very small in size, often not exceeding 2cm. We report an exceptional case of giant insulinoma initially revealed by a pseudo-polycythemia in an 80-year-old man. He had been treated for hypertension for a few months. Routine biological investigations showed elevated hematocrit and haemoglobin, suggesting Vaquez disease. History taking revealed recent episodes of nocturnal agitation. On admission, he had reddish skin with a suspected enlarged spleen, but total blood volume was normal. Imaging studies showed a voluminous tumor located between the pancreas and the spleen. The presence of an insulinoma was confirmed on the basis of an elevated level of proinsulin at the time of an asymptomatic episode of hypoglycemia. Spleno-pancreatectomy was performed. Histopathological examination revealed a malignant, well-differentiated neuroendocrine malignant tumor.

This study shows the contribution of MRI to diagnosis and follow up of the cerebral lesions in neuro-lupus. Ten cases of neuro-lupus have been reported. The affected patients are 7 women and 3 men having an average age of 34 years. For 8 patients, MRI revealed lesions that were not detected using computed tomography. These lesions were high signal zones on T2, scattered with predilection to deep and sub-cortical cerebral white matter. MRI has also shown cortical and sub-cortical cerebral atrophy for 3 cases, cortical and deep infarcts for 2 cases and both atrophy and infarcts in one case. The MRI diagnosis was normal for the two remaining patients. MRI contributes to the diagnosis confirmation and localization of the cerebral lesions and to the follow-up. In spite of MRI sensitivity, the detection of these lesions remains non specific to neuro-lupus. New methods of exploration as the spectroscopy by magnetic resonance and the cerebral scintigraphy with Technetium 99m could be very helpful in diagnosis of cases for which MRI has shown limitations.

We report 3 cases of extramedullary hematopoiesis with a sacrococcygeal, right obturatory hole and paravertebral dorso lumbar locations respectively in 30, 34 and 58 years old patients (two males and woman). A medullary tuberculosis was discovered in one case, a beta thalassemia in the second, and the third was classified as idiopathic. Radiologic picture often permitted to evoke the diagnosis on the aspect of a well limited multilocular tumor especially when there is a paravertebral location as in our third patient. Fine needle punction cytologic examination can permit the diagnosis and avoid surgery, except if complications occur.