Epidemiological, clinical, and therapeutic characteristics of Behçet's disease: a monocentric study in Tunisia.

INTRODUCTION: to describe the epidemiological, clinical, therapeutic and evolving characteristics of Behçet´s disease and identify prognostic factors. METHODS: we have realized a retrospective, single-center study, conducted over a period of 26 years and including 130 patients presenting Behçet´s disease and hospitalized in an Internal Medicine Department. RESULTS: the mean age of the Behçet´s disease at onset was 30.3 ±8.8 years and that at diagnosis was 34.6 ±9.4 years. The sex ratio (male/female) was 2.5. The mean delay of diagnosis was 53.5 months. Oral aphthosis was constant. The frequency of the manifestations was: genital aphtosis 71.5%, pseudofolliculitis 84.6%, erythema nodosum 11.5%, positive pathergy test 50%, ocular disease 36.9%, venous thrombosis 30%, arterial disease 4.6%, joint damage 30.8%, neurological disease 19.2% and digestive disease 0.8%. The male gender was significantly associated with ocular involvement (p =0.02), venous disease (p =0.01) and occurrence of relapses (p =0.01). The mean follow up was 68.5 ± 77.3 months. The poor survival prognostic factors were male gender, ocular involvement, venous disease, cardiovascular disease, a duration of follow up ≤12 months and a diagnostic delay ≤ 24 months. Conclusion: improving the prognosis of Behçet´s disease requires a shortening of the time to diagnosis, multidisciplinary collaboration, intensive treatment of functional threats, regular monitoring, and patient adherence.

Salmonella osteomyelitis complicating aseptic osteonecrosis: Don't forget about the antiphospholipid syndrome.

Osteonecrosis in antiphospholipid syndrome is a diagnostic challenge for clinicians. Early diagnosis and intervention are important for better prognosis.

Myxofibrosarcoma of the leg: A diagnostic challenge.

We should keep in mind slowly growing malignancies when the lesion is located close to the synovial regions of the extremities. The diagnosis of certainty of myxofibrosarcoma is histological and is based on the demonstration of the myxoid matrix, fibroblastic cells with a curvilinear arrangement of the vessels.

Arterial Hypertension in Systemic Lupus Erythematosus: About 40 Cases.

The aim of this study was to determine the prevalence and the etiologic profile of hypertension (HTN) in systemic lupus erythematosus (SLE). A retrospective analysis was performed on 153 patients with SLE who attended our center for 16 years from January 2000 to December 2016. The diagnosis of SLE was established according to the classification criteria of the American College of Rheumatology in 1990. The prevalence of HTN in patients with SLE was 26.1% (40/153)' with an average delay of appearance of 21 months. There were 37 women and three men with a mean age of 46' eight years (20-70). HTN was associated with lupus nephritis (n = 8)' other renal impairments (n = 6)' and corticosteroid treatment (n = 20). Essential HTN was found in six cases. Cardiovascular factors associated with HTN were: diabetes (n = 14)' sedentary life (n = 15)' obesity (n = 12)' and dyslipidemia (n = 8). Main clinical manifestations associated with HTN were: arthralgia/arthritis (24 cases)' cutaneous involvement (22 cases)' and hematological manifestations (16 cases). Anti-phospholipid syndrome was found in 12 cases. Coronary artery disease' arteritis of lower limb' and transient ischemic attacks complicated the course of HTN in six patients. Angiotensin-converting-enzyme inhibitors were the most commonly used drug for treatment in this group. HTN was frequently associated with corticosteroid treatment in this study. We feel that the use of corticosteroids should be avoided as far as possible in all patients with SLE.

Predictive factors of the lupus nephritis in a Tunisian cohort.

INTRODUCTION: Renal involvement is a common and serious manifestation of systemic lupus erythematosus (SLE) and it is life-threatening. AIM: To identify the predictive factors of the lupus nephritis (LN). METHODS: A descriptive, analytical, single-centre, retrospective study of 115 patients with SLE (ACR 1997) was carried out in an internal medicine department for a period of 20 years from 1997 until 2017. LN was diagnosed by proteinuria ≥ 0.5 g /24h and / or urine sediment abnormality. Eighty patients did not have kidney damage during the follow-up and thirty-five developed renal involvement after SLE diagnosis. RESULTS: The univariate analysis retained several epidemiological, clinical and biological correlation parameters, according to the development of lupus nephritis, statistically significant. They are as follows, the male gender, the age of diagnosis of SLE less than or equal to 34 years, the presence of malar rash by the time of SLE diagnosis, naso/pharyngeal ulcerations, leucopoenia, positivity of anti-Sm antibody and low C3 complement.The multivariate analysis had found that age less than or equal to 34 years at the diagnosis of the SLE was the only predictive factor of the onset of LN (OR=5.1 and HR=3.4). CONCLUSION: Given the seriousness of the pathology and the complexity of its management, LN should be detected as soon as possible and must be treated appropriately by selecting the lupus population at risk for developing a serious LN.

[Syndrome d'activation macrophagique: une série de 11 cas tunisiens]. / Hemophagocytic syndrome: a study of 11 patients in tunisian population.

BACKGROUND: Hemophagocytic syndromes are mostly associated with underlying pathology, they can reveal: immunodeficiency, infections, hemopathies, cancers and auto-immune diseases. AIM: to investigate clinical, biological features, outcome characteristics and underlying pathology of Tunisian patients with hemophagocytic syndromes. METHODS: A retrospective study of patients with hemophagocytic syndromes admitted in an internal medicine department in Tunis over the period 2009-2012. RESULTS: There were 11 patients included, 4 men and and 7 women. Mean age was 47,3years. Purpura was observed in 3 cases, hepatosplenomegaly in 6 cases and peripheral lymph nodes in 3 cases. Hemorrhagic complication was noted in 2 cases. There were an increase of inflammatory indices in all the cases. Eight patients had pancytopenia and 3 had bicytopenia. Six patients developed intravascular dissiminated coagulation. High levels of triglyceridemia was noted in 5 cases and an hyperferritinaemia in all cases. Cytological examination of bone marrow confirmed hemophagocytosis in ten cases. Hemophagocytosis was associated to infectious diseases in 6 cases, there were 2 cases of leishmaniasis, septicemia to E.Coli and staphylococcus aureus and lymph nodes tuberculosis with HIV. We also noted a case of lymphoma, myelodysplasic syndrome, rheumatoid polyarthritis, adult onset still's disease. In one patient, the origin of hemophagocytosis remained indefinite. Visceral leishmaniose were treated by Glucantime®, adult Still's disease by corticosteroids and méthrotrexate®, lymphoma by chemotherapy. In lymph nodes tuberculosis, there were good evolution. Six patients died. CONCLUSION: Hemophagocytic syndrome is a rare and life-threatening disease. Pejorative prognosis requires an early therapy with etiological treatment.