[Renal involvement in ankylosing spondylitis: concerning 210 cases]. / Les manifestations rénales de la spondylarthrite ankylosante: à propos de 210 cas.

PURPOSE: To define the epidemiology, clinical, biological and histological features of renal diseases in ankylosing spondylitis, ginving special attention to unusual forms. METHODS: We retrospectively reviewed the medical record of 28 cases with renal involvement among 210 cases of ankylosing spondylitis seen over a 27 year period who met the Amor criteria. RESULTS: Twenty-eight of 210 patients (13,3%) presented one or more signs of renal involvement: macroscopic hematuria (4 patients), microscopic hematuria (8 patients), proteinuria (15 patients), nephrotic syndrome (6 patients), decreased renal function (13 patients). Secondary renal amyloidosis and nephrolithiasis (8 patients) were the most common cause of renal involvement in ankylosing spondylitis followed by IgA nephropathy (3 patients). CONCLUSION: The funding of renal abnormalities in 13,3% of our patients suggests that in this illness evidence of renal involvement should be actively investigated in ankylosing spondylitis.

[Sternal metastasis revealing hepatocarcinoma]. / Métastase sternale révélatrice d'un hépatocarcinome.

INTRODUCTION: Bone metastasis from hepatocarcinoma are rare, their elective seats are the ribs, the vertebra and rarely the sternum. We report a case of a sternal metastasis which makes discovery of a hepatocarcinoma. EXEGESIS: A 64 year-old man, alcoholic with a previous history of jaundice who developed since 1999 an anterior chest tumor with excellent clinical condition. Laboratory examination showed cytolysis, cholestasis, positive antihepatitis C virus antibodies and elevated serum alphafetoprotein level. Standard radiography and computed tomography of the chest showed an osteolytic lesion of the sternum spread to the adjacent soft tissues and voluminous right hepatic lesion. Pathologic examination of the sternal tumor concluded to a differential adenocarcinoma. Etiologic investigations to find the primitive tumor were negative. Operative procedure was not possible in consideration of the infiltration of the tumor and its situation near the main blood vessels. Radiotherapy gives rise to partial regression of the tumor. A second reading of the tumor biopsy established the diagnosis of metastasis from a hepatocellular carcinoma. The patient died 22 months after the appearance of the sternal metastasis. CONCLUSION: Hepatocarcinoma is rarely disclosed by a sternal metastasis, our case-report is particular by its prolonged survival and the good clinical condition during its follow up.

[Adult Still's disease: study of a series of 11 cases]. / La maladie de Still de l'adulte: étude d'une série de 11 cas.

Adult Still's disease is a systemic disease of unknown etiology. We report a retrospective study of 11 cases (9 females and 2 males) of adult Still's disease collected during 25 years. The mean age was 36 years. Fever, arthritis and skin rash was constant. Adenopathies and splenomegaly were observed in 2 patients. The laboratory findings was characterized by a constant inflammatory syndrome and leucocytosis. Hypertransaminasemia and hyperferritinemia were observed respectively in 7 cases and 3 cases. Corticosteroids were prescribed in all patients. Methotrexate was administered in 3 patients. Outcome was favorable in 10 cases, death incurred in one patient, secondary to acute hepatitis.

[Neurological manifestations in Behcet's disease. Forty observations in a cohort of 300 patients]. / Manifestations neurologiques de la maladie de Behçet.

PURPOSE: To analyse and discuss neurological involvement of Behçet' disease and therapeutical possibilities giving special attention to unusual forms. METHODS: We retrospectively reviewed the medical records of 40 cases with neurological manifestations among 300 cases of Behçet's disease seen over a 20 years period who met the international study group of Behçet's disease criteria. RESULTS: Central nervous system involvement was found in 39 patients and peripheral nervous involvement in one. Dominant symptoms in 40 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (27 cases), intracranial hypertension (9 cases), psychic disturbances (2 cases), and polyneuritis of the lower limb (1 case). Headache and focal deficits were the major presenting signs (87%). Cerebral venous thrombosis was identified in 8 patients (6 certain and 2 probable). These could be differentiated from meningoencephalitis by their symptomatology predominaly that of intracranial hypertension. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level cerebral CT scan, performed in 20 patients, was normal in 30% of cases. MRI performed in 2 patients, showed in one case a tumor like lesion. CONCLUSION: Neurobehçet's disease can be classified into 2 clinical aspects: meningoencephalitis and cerebral venous thrombosis. Treatment is aimed initially at reducing the inflammation with corticosteroids and preventing relapse with adjunction of an immunosuppressor.

[Pseudotumoral neurobehçet: a case report]. / Une forme pseudotumorale d'un neurobehçet.

Neurological involvement occurs in 5.3% to 30% of patients with Behçet's disease. Neurological manifestations can be secondary to direct central nervous system involvement (encephalitis, encephalomyelitis) or vascular angeitis (essentially cerebral venous, thrombosis and, rarely, intracranial aneurysms). Neurological pseudotumoral presentation is rarely reported. We report a case of 26-year-old woman suffering from left hemiplegia. MRI was performed and showed pseudotumoral lesion in the protuberance and the right cerebral pedicule. Oral and genital aphtous ulcers were found. HLAB51 was positive. The patient improved with steroid therapy.

[Tumorous calcinosis in hemodialysis: anatomo-clinical study apropos of 3 cases]. / La calcinose tumorale des hémodialysés: étude anatomoclinique: à propos de trois cas.

Three patients (2 females, 1 male) with a mean age of 51.4 years receiving long term hemodialysis affected by tumoral calcinosis were analysed. Clinical, radiological and pathological features were evaluated and pathogenic were reviewed. The joints involved int the cases presented in this report were the hip shoulder and finger. The lesions were bilateral in shoulder. An increased calcium-phosphorus product (Ca x P) was observed in all patients with secondary hyperparathyroidism in one case. Surgery was carried out in all patients. No relapse of the tumoral calcinosis was observed after surgery. The most important pathogenic factor involved in uremic tumoral calcinosis is an increase in calcium-phosphorus product (Ca x P) not necessarily related to hyperparathyroidism. Therefore, maintaining the calcium x phosphate product within the normal range appears to be the most important factor ito prevent the appearance of uraemic tumoral calcinosis.

[Arterial aneurysm in Behçet's disease. Report of 5 cases]. / Anévrismes artériels au cours de la maladie de Behçet. A propos de 5 observations.

Behçet's disease is a systemic vasculitis generally involving the venous system. Arterial manifestations, usually aneurysm or more rarely occlusion, are less common. We analysed 5 cases of Behçet's disease with arterial aneurysm complications. There were 4 men and 1 woman, mean age 34.6 years. Mean delay to arterial complications was 7.8 years after the first sign of the disease. Four patients showed evidence aneurysms of the pulmonary arteries. One patient developed an aneurysm of the aortic and iliac artery. Phlebitis was associated with arterial involvement in 4 patients. Combined corticosteroid and cyclophosphamide therapy enabled regression of pulmonary aneurysms in 2 patients. One patient was operated, outcome was favorable. Histology showed fragmentation of the media associated with vasculitis. Two patients with pulmonary aneurysms died of massive hemoptysis.