5 research outputs found
Fulminant lupus pneumonitis complicating systemic lupus erythematosus in the elderly
Abstract Fulminant lupus pneumonitis is a rare complication of SLE. We report a case of 75âyearsâold male patient with SLE who developed pneumonia and severe respiratory failure requiring mechanical ventilation. Refractory respiratory distress complicating noninfectious fulminant lupus pneumonitis did not respond to methylprednisolone and intravenous immunoglobulin treatment
B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease
Remitting-RelapsingMultiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuroinflammatory disorders leading to neurological damage. Herein, we investigated in these patients the IL-10-producing cells during the early stages of these disorders. Cellular and molecular investigations were carried out on treatment naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Our findings demonstrate that CSF-B cells from NBD patients, but not RRMS, are the major source of intrathecal IL-10 as compared to T-CD4 cells. Moreover, we showed a lower expression of TGF-β and IL35, in the CSF cells of NBD patients as compared to the control group. Specific in vitro CpG stimulation of peripheral blood B cells from NBD patients resulted in a concomitant early mRNA expression of IL6 and IL10 but was limited to IL10 for RRMS patients. Furthermore, mRNA expression of IL-6 and IL-10 receptors was assessed and intriguingly IL6ST receptor subunit was significantly lower in NBD CSF, but not RRMS while IL10RB was increased in both. Deciphering the role of increased IL-10-producing B cells and IL10RB despite relapsing disease as well as the discordant expression of IL6 and IL6ST may pave the way for a better understanding of the pathophysiology of these neuro-inflammatory disorders
B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçetâs Disease
Remitting-RelapsingMultiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuroinflammatory disorders leading to neurological damage. Herein, we investigated in these patients the IL-10-producing cells during the early stages of these disorders. Cellular and molecular investigations were carried out on treatment naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Our findings demonstrate that CSF-B cells from NBD patients, but not RRMS, are the major source of intrathecal IL-10 as compared to T-CD4 cells. Moreover, we showed a lower expression of TGF-ÎČ and IL35, in the CSF cells of NBD patients as compared to the control group. Specific in vitro CpG stimulation of peripheral blood B cells from NBD patients resulted in a concomitant early mRNA expression of IL6 and IL10 but was limited to IL10 for RRMS patients. Furthermore, mRNA expression of IL-6 and IL-10 receptors was assessed and intriguingly IL6ST receptor subunit was significantly lower in NBD CSF, but not RRMS while IL10RB was increased in both. Deciphering the role of increased IL-10-producing B cells and IL10RB despite relapsing disease as well as the discordant expression of IL6 and IL6ST may pave the way for a better understanding of the pathophysiology of these neuro-inflammatory disorders
A Tunisian patient with CLCN2ârelated leukoencephalopathy
Abstract CLCN2ârelated leukoencephalopathy (CC2L OMIM#: 615651) is a recently identified rare disorder. It is caused by autosomal recessive mutations in the CLCN2 gene and leads to the dysfunction of its encoded CLCâ2 chloride channel protein with characteristic brain MRI features of leukoencephalopathy. We report the first Tunisian patient with clinical features of ClCNâ2ârelated leukoencephalopathy. A 54âyearâold female with a family history of leukemia, male infertility, motor disability, and headaches who initially presented with a tensionâtype headache and normal physical examination. At the followâup, she developed mild gait ataxia and psychoâcognitive disturbances. A previously reported homozygous NM_004366.6(CLCN2):c.1709Gâ>âA (p.Trp570Ter) stop gained mutation was identified. This report expands the knowledge related to CC2L and highlights the clinical features in affected individuals of African descent
Value of inferior vena cava collapsibility index as marker of heart failure in chronic obstructive pulmonary disease exacerbation
Abstract Introduction Inferior vena cava (IVC) diameter variability with respiration measured by ultrasound was found to be useful for the diagnosis of heart failure (HF) in ED patients with acute dyspnea. Its value in identifying HF in acute exacerbation of chronic obstructive pulmonary disease exacerbation (AECOPD) was not specifically demonstrated. Objective To determine the value of ÎIVC in the diagnosis of HF patients with AECOPD. Methods This is a prospective study conducted in the ED of three Tunisian university hospitals including patients with AECOPD. During this period, 401 patients met the inclusion criteria. The final diagnosis of HF is based on the opinion of two emergency experts after consulting the data from clinical examination, cardiac echocardiography, and BNP level. The ÎIVC was calculated by two experienced emergency physicians who were blinded from the patientâs clinical and laboratory data. A cut off of 15% was used to define the presence (<â15%) or absence of HF (â„â15%). Left ventricular ejection fraction (LVEF) was also measured. The area under the ROC curve, sensitivity, specificity, and positive and negative predictive values were calculated to determine the diagnostic and predictive accuracy of the ÎIVC in predicting HF. Results The study population included 401 patients with AECOPD, mean age 67.2 years with male (68.9%) predominance. HF was diagnosed in 165 (41.1%) patients (HF group) and in 236 patients (58.9%) HF was excluded (non HF group). The assessment of the performance of the ÎIVC in the diagnosis of HF showed a sensitivity of 37.4% and a specificity of 89.7% using the threshold of 15%. The positive predictive value was 70.9% and the negative predictive value was 66.7%. The area under the ROC curve was 0.71(95%, CI 0.65â0.76). ÎIVC values were not different between HF patients with reduced LVEF and those with preserved LVEF. Conclusion Our results showed that ÎIVC has a good value for ruling out HF in ED patients consulting for AECOPD