Syrine Bellakhal

Characteristics of neurological manifestations of Behcet's disease: A retrospective monocentric study in Tunisia

OBJECTIVEnThe aim of the present study was to analyze demographic, clinical and genetic features of Behçets disease patients with neurological involvement through a monocentric study of a homogenous group of hospitalized patients observed in the same department and to compare them with those of other ethnic and geographic groups.nnnMETHODSnFour hundred and thirty Behçets disease (BD) patients were retrospectively studied. Diagnosis of BD was made according to the international study group for Behçets disease criteria. Patients with neurological findings suggestive of involvement of the nervous system by BD were further studied according to clinical examination, laboratory tests and neuroradiological investigations.nnnRESULTSnNeurological involvement was observed in 121 patients (28.1%). The mean age at neuro-Behçets disease (NBD) onset was 29.7 years. Average disease duration of BD before neurological manifestations onset was 6.4 years. Male to female ratio was 1.8. Of the 121 NBD patients, parenchymal involvement occurred in 74 patients (61%). Among them 26 (21.4%) presented with brainstem involvement, 24 (19.8%) with hemispheric involvement and 2 (1.6%) with spinal cord involvement. Non-parenchymal NBD occurred in 47 patients (39%). Involvement of the main vascular structures (Vasculo-NBD) was the most common non-parenchymal NBD lesion found in 35 patients (28.9%) consisting of cerebral vein thrombosis (CVT) in 24 cases and cerebral arterial thrombosis in 11 cases. Forty-nine (40.5%) patients with NBD have been followed-up for a median of 3 years (range 1-19 years). Forty-one of them recovered well without significant residual disability, 5 patients made no improvement and are left with severe neurological impairments and 3 died. Male gender and CNS parenchymal lesions occurrence were significantly associated with a poorer prognosis.nnnCONCLUSIONnClinical and epidemiological features of NBD are various. In our Tunisian cohort of NBD patients the main characteristic features were male predominance, a relatively high prevalence of CVT, a low prevalence of intra-cranial hypertension and a significant lower frequency of HLA-B51 haplotype.

Efficacy and safety of rituximab in systemic sclerosis: French retrospective study and literature review

OBJECTIVEnTo describe safety and efficacy of rituximab in patients with systemic sclerosis.nnnMETHODSnWe included 13 patients with systemic sclerosis treated with rituximab and pooled with 40 additional patients from the literature. SSc rituximab untreated patients were matched to rituximab treated ones.nnnRESULTSnThirteen patients who received rituximab and 26 rituximab-untreated patients were included. In comparison to 26 patients who did not received rituximab, FVC changes were not significantly different, whereas DLCO improved in 13 patients who received rituximab (0 [-4; 4] vs loss of -7 [-19; 0]; p=0.05). Considering 7 rituximab treated and 14 untreated diffuse SSc, FVC was improved during the 24 [12; 46] months of follow up in dSSc who received rituximab (gain of 12 [7.5:14] % vs loss of 1.5 [-16.8; 2.5], (p=0.003)). Pooled analysis of 53 patients (40 literature patients and 13 from personal series) showed significant improvement of median mRSS from 18 [8; 32] at baseline to 9 [4; 18] at M6 (p=0.007), 13 [8; 18] at M12 (p=0.008) and 10 [4; 16] at the last follow-up (p=0.0002). FVC increased from 71% [66; 80] at baseline to 84% [75; 90] at M12 (p=0.001). DLCO increased from 58% [39; 65] at M0 to 63% [53; 78] at M12 (p=0.04).nnnCONCLUSIONnOur personal data and pooled literature analysis suggest the efficacy of rituximab in the subset of diffuse SSc in particular in skin and interstitial disease involvements. The safety of rituximab seems to be reasonable and similar to previous data in other autoimmune diseases.

C0393 Risk factors and prevalence of venous thromboembolism in internal medicine: A retrospective study of 806 patients

disease. We aim to study the prevalence and the cancer types in patients with deep venous thrombosis (DVT). Methods: A retrospective study of patients with established DVT confirmed with Doppler ultrasound. Cancer was suspected according to clinical and biological signs and confirmed by radiological or histological examination. Results: One hundred and eight among the 806 patients with DVP had cancer-associated thrombosis (13.39%) Themean age at the diagnosis of DVT was 63.5 years (20–87 years). They were 65 men and 43 women. Urological cancers were the most frequently associated with DVT, they were found in 33 cases (30,5%). In others cases, cancer location was the digestive tract in 19 cases (17.5%), gynecological cancers in 18 cases (16%), lung cancer in 12 cases (11, 1%), hematological malignancy in 9 cases (8,3%) brain cancer in 8 cases (7,4%) and head and neck cancers in 5 cases (4,6%). In 50% of cases thrombosis had revealed the cancer. Comment: Cancer patients are well-known to be at increased risk of DVT. However the risk varies widely between patients. Others comorbidities of patients, cancer stage and treatment modalities plays an important role in thrombosis. Thrombotic manifestations are common and potentially lethal in patients with active cancer, Extensive screening for cancer in patients with idiopathic thromboembolism help to detect the majority of malignancies.