Thouraya Ben Salem

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

OBJECTIVE The 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. METHODS Four 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. RESULTS Neurological 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. CONCLUSION Clinical 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.

Contribution of Hla-B51 in the susceptibility and specific clinical features of Behcet's disease in Tunisian patients

BACKGROUND Many researchers have tried to investigate the association of HLA-B51 with the severity and the clinical features of BD with conflicting results. METHODS We aimed at investigating the association of HLA-B51 with demographical and clinical manifestations as well as the severity of BD, by studying 178 native Tunisian BD patients, fulfilling the International Study group criteria for the BD classification recruited from the Department of Internal Medicine, Rabta Hospital in Tunis and compared with 125 native Tunisian healthy age and sex matching volunteers. RESULTS According to our findings, the frequency of HLAB 51 was significantly higher in BD patients than in controls (p<0.001). Positive pathergy test (PPT) (p = 0.01) and retinal vasculitis (p = 0.045), were significantly more frequent in HLA B51(+) patients, while the frequency of arterial aneurysms (p = 0.009) and neurological involvement, especially the parenchymal involvement (p<0.001), were significantly and clearly higher in HLA B51(-) patients. The patients without HLA B51 had a significantly less severe disease (p = 0.001). Discussion/conclusion We conclude that HLA B51 is a predisposing marker for BD in our population as in most ethnic groups. It seems to be associated with a subgroup of BD patients characterized by a higher frequency of ocular involvement and PPT, but a lower frequency of arterial aneurysm and neurological involvement, and a less severe disease course.

Pleural myelomatous involvement in multiple myeloma: five cases.

Pleural myelomatous involvement in multiple myeloma (MM) is rare, occurring in less than 1% of cases. We retrospectively studied five cases of patients with MM who developed myelomatous pleural effusions. Three men and 2 women with a mean age of 61 years presented with myelomatous pleural effusion. The pleural fluid electrophoresis revealed a peak of IgG in three cases, of IgA in one case, and of lambda light chains in one case, which were identical to that in the sera of the patients. Detection of typical plasma cells in pleural fluid cytology was contributive, and histologic confirmation by pleural biopsy was positive in four cases. Treatment consisted of chemotherapy. The clinical outcome was initially good, but relapses occurred in all cases early and were complicated by fatal infections. Myelomatous pleural effusion is a rare affection. It is usually a late complication associated with poor prognosis.

Central Retinal Vein Occlusion Revealing Coelic Disease

Introduction Thrombosis has been widely reported in coeliac disease (CD) but central retinal vein occlusion (CRVO) is rarely described. Case presentation A 27-year-old woman presented with acute visual loss and was diagnosed with CRVO. Her protein S and protein C levels were low and CD was diagnosed on the basis of endoscopic, immunological and histological results. A gluten-free diet resulted in favourable evolution. Conclusion CD should be considered in young patients with thrombosis, especially if in an unusual location. Treatment is based on a gluten-free diet. LEARNING POINTS Coeliac disease (CD) should be considered in young patients with central retinal vein occlusion (CRVO). Several mechanisms can cause thrombosis in CD. CRVO in CD is often reversible with a gluten-free diet.

Primary Sjögren’s syndrome complicated by anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulonephritis

Ocular and oral dryness are the hallmark of Sjögrens syndrome (SS). However, SS can be associated with a variety of complications, affecting organs such as the liver, kidneys, lungs, muscle, and nervous system. Renal involvement has been usually in the form of tubulointerstitial nephritis. However, glomerulonephritis is rare in primary SS. We report three clinical cases of SS with anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulo-nephritis treated with prednisone and cyclophosphamide, with favorable outcome.

Deep Venous Thrombosis in Behçet's Disease: A Retrospective Study of 430 Tunisian Patients

Behçet’s disease (BD) is a chronic inflammatory disorder characterized by an association of recurrent oral aphthae and other manifestations such us genital ulcers, skin lesions, arthritis, uveitis and thrombophlebitis. It may also involve central nervous and gastrointestinal systems [1,2]. The etiology and pathogenesis of BD are not clear, but are presumed to be multifactorial, implicating genetic, infectious and immunologic factors. The predominant histopathological feature in BD is vasculitis with singular properties among other systemic vasculitis manifestations, as it can involves all veins, arteries and vessels of all sizes [3]. The prevalence of vascular involvement in BD varies from 6 to 40% according to the studied population [4,5]; deep venous thrombosis (DVT) are the most frequent vascular manifestation, seen in 6.2 to 33 % cases of BD [5-7]. Pathogenesis of vasculo-BD is still not well established, but it seems that vascular endothelial lesion is the major factor [8].

Fatal Myocarditis in a Male with Systemic Sclerosis

Cardiac involvements in systemic sclerosis have a poor outcome. We report here a case of 28 year-old-man with history of systemic sclerosis developing a fatal myocarditis. Initially patient has disabling joint manifestation. Systemic sclerosis is diagnosed after progressive development of cutaneous, pulmonary, and gastrointestinal manifestations. One year after systemic sclerosis diagnosis, our patient develops heart failure signs when he is already treated with methotrexate (15 mg/week) and coticosteroids (15 mg/day). Echocardiography show global hypokinesia with low left ventricular ejection fraction (32%). Natriuretic peptides and troponin levels are high. Anti-centromere and anti-SRP antibodies are positive. Corticosteroids dose is increased to 1 mg/kg/day, and methotrexate was given 25 mg/week without improvement. Cyclophosphamide was started but patient died within six months. Despite immunosuppressive therapy, myocarditis outcome is poor since patient was in heart failure at presentation, and has anti-SRP antibodies.

0424: Cardiovascular involvements in Takayasu arteritis in Tunisia: clinical study of 43 cases

Introduction Takayasu arteritis (TA) is a rare systemic vasculitis. The aim of this study was to describe cardiovascular involvements in Tunisian patients with TA. Patients and Methods Retrospective study of patients with TA (American College of Rheumatology criteria) admitted in an internal medicine department from 1992 to 2015. Results Forty three patients were identified. Sex ratio M/F was 0.16. Mean age of Takayasu first manifestation was 36.2 years. Eleven patients complained of chest pain and 76.74% of intermittent limb claudicating (upper and lower limbs). 35 patients had pulse disturbance mainly radial pulse (n=28) and 23 patients had artery hypertension. Vascular murmur was noted in 62.79% of cases. Aortic murmur was systolic in 6 cases and diastolic in 3 cases (aortic valve regurgitation). Electrocardiogram showed abnormalities in 9 cases: 1 right bundle branch block, 2 complete left bundle branch block, 3 left ventricular hypertrophy, 2 atrial fibrillation and T negative wave from V3 to V6. Biological inflammatory syndrome was found in 32 patients. Aortic arch, its branches (n=33) and left supra clavicular artery (n=24) were the most common involvements. Thirty-three patients received corticosteroids and 13 had immunosuppressant drugs. Eighteen patients received antiplatelet agents and 6 underwent surgery. Angioplasty was performed in 5 patients. Outcome was good in 14% of cases. Three patients died (stroke). Conclusion Our study confirmed the female predominance and the frequency of hypertension. These results are common to the North African data previously published and confirm the particularity of TA in our region, which is different from the Asian one.

0201: Cardiac involvement in patients with sarcoidosis

Introduction Sarcoidosis is a systemic granulomatous disease of unknown etiology. Cardiac involvements in patients with sarcoidosis are rare with an estimated prevalence of 7%. These serious involvements are hard to diagnose at early stage of disease. Patients and Methods We performed a retrospective study of patients’ files diagnosed with sarcoidosis in the Internal Medicine Department of Rabta University Hospital in Tunis. The diagnosis of sarcoidosis was based on clinical, paraclinical and histological criteria. Cardiac involvements were confirmed by the electrocardiogram and echocardiography. Results Cardiac involvement (CI) was found in seven patients among 138 with sarcoidosis (5%). They were 6 females and one male with a mean age at diagnosis of 49 years. Electrical disorders such as bundel branch block were seen in two cases. First degree atrioventricular block was noted in two cases. Ventricular hypertrophy was check out in two cases by electrocardiography and ultrasound. Mitral regurgitation with a flutter was noted in one patient and pericardial effusion with a normal ventricular function in another case. Only one patient was clinically symptomatic, he complained of palpitations. Thallium 201 scintigraphy was performed in one case and objectived reversible ischemia of the inferior and latero-inferior walls of myocardium. Other manifestations of sarcoidosis were dominantly associated to CI such as lung disease, which was found in all patients. Skin and neurological involvement were observed, each in three cases. Hepatosplenic involvements were noted in two patients and otorhinolaryngology involvements in one case. All patients received initially high dose of corticosteroids. Four of the patients had a good response to treatment and three were lost to follow up. Conclusion Cardiac involvements in sarcoidosis are rare but serious, an early diagnosis and treatment with corticosteroid may rapidly improve patients.

0410: Cardiovascular manifestations in antiphospholipid syndrome

Introduction Antiphospholipid syndrome (APLS) is defined by the occur-rence thrombosis, and/or obstetric morbidity associated with the presence of antiphospholipid antibodies. Cardiovascular manifestations of APLS are variable and some of them can be life threatening. Patients and Methods Retrospective study of patients with APLS (diagnosed according to Sidney classification criteria of 2006) in an Internal medicine department over a period of 14 years. Results Thirty-four patients with APLS had cardiovascular involvements (66.6%); 30 females and 4 males, with a mean age of 40.5 years (24-74 years). Cardiovascular involvements revealed the disease in 53% of cases. Venous thromboses were most frequent manifestation: deep-vein thrombosis (DVT) (12 cases), pulmonary embolism (5 cases), cerebral venous thrombosis (1 case), inferior vena cava thrombosis (1 case), portal vein thrombosis (1 case), superficial venous thrombosis (1 case) and central retinal vein occlusion (2 cases). Arterial thromboses were noted in 12 patients: stroke (9 cases), upper limb artery, renal artery and occlusion of central retinal artery (each in 1 patient). Acute coronary artery thrombosis was observed in one patient (myocardial infarction). Pericarditis was noted in 3 cases, valvular disease in 2 cases (an aortic and a mitral stenosis), and myocarditis in 1 patient. Anticardiolipin and anti-B2 glycoprotein I antibodies were positive in 47.1% and 52.8% of cases respectively. Anticoagulant therapy was prescribed in thrombotic cases. Pericarditis were treated with corticosteroids and patient with myoarditis had both corticosteroid and immunosuppressant agent. Conclusion DVP were the most frequent vascular manifestation in patients with APLS. Cardiac involvements are rare but serious. They can affect all parts of the heart but cardiac valvulopathy and coronary artery thrombosis are the most frequent.