Serum cystatin C is associated with kidney function but not with cardiovascular risk factors or subclinical atherosclerosis in patients with Systemic Lupus Erythematosus.

Cystatin C (CysC) is a protein considered as an excellent marker of renal function, and it has been suggested as an independent predictor of cardiovascular (CV) risk. We evaluated the association of serum CysC with renal function, CV risk factors, inflammation, and subclinical atherosclerosis in Systemic Lupus Erythematosus (SLE) patients. Sixty-one SLE female patients were selected according to estimated glomerular filtration rate (GFR) > 60 ml/min/1.73m2. Renal function parameters, SLE specific factors, CV risk factors, and inflammatory markers were assessed. Subclinical atherosclerosis was assessed by measuring the carotid-femoral pulse wave velocity (PWV) by Doppler velocimetry. Serum CysC concentration was measured using a particle-enhanced immunonephelometric assay that established 0.59-1.01 mg/l as reference values. Patients with high CysC showed significantly altered creatinine, microalbuminuria, and GFR in addition to a significant higher presence of traditional CV risk factors such as arterial hypertension (p < 0.001), metabolic syndrome (p < 0.001), hypertrigliceridemia (p < 0.001), tobacco habit (p < 0.05), and a strong association with arterial stiffness (p = 0.017). Positive correlation between CysC, homocysteine (r = 0.511; p < 0.001) and fibrinogen levels (r = 0.304; p < 0.02) were also observed. A significantly higher SLICC/ACR score was related to high CysC level (p = 0.011), together with higher endothelin-1 and lower TNF serum concentration (p < 0.005). Considering only patients without any renal impairment (microalbumin/creatinine <30 mg/g), no association between CysC level and CV risk factors, arterial stiffness, or SLE-related factors was found. Serum CysC is a good marker of renal function in SLE patients, but it is not independently associated with cardiovascular risk factor or subclinical atherosclerosis.

Genotypic distribution of common variants of endosomal toll like receptors in healthy Spanish women. A comparative study with other populations.

Genetic variants of endosomal toll like receptors (TLR) have been associated with many infectious, autoimmune and inflammatory diseases, but few studies have been reported in the Spanish population. The aim of this study was to describe the allelic and genotypic distributions of some common nucleotide substitutions of endosomal TLRs in healthy Spanish women and to compare them with those already published in other population groups. Nine substitutions were analysed in 150 DNA samples from 150 Spanish, non-related healthy females: TLR3 rs3775291 and rs5743305; TLR7 rs179008 and rs5743781; TLR8 rs3764880 and TLR9 rs187084, rs5743836, rs352139 and rs352140. Genotyping was carried out by real time PCR and melting curve analysis in a LightCycler 480. A systematic review was performed in order to compare the genotypic distributions in our cohort with those previously published in other population groups. The comparative study was performed with the two tailed Fisher's test or the Yates continuity correction for the Chi-square test when appropriate. No homozygotes for rs5743781 in TLR7 were found, and rs352139 and rs352140 of TLR9 were in strong linkage disequilibrium. Genotype distributions in endosomal TLR are similar to other Spanish series previously reported. As expected, most differences were found when comparing our distributions with Asiatics, but differences were also found with other Caucasian populations. Since there are significant variations in genotypic distributions of TLRs in both interracial groups and within the same ethnic group, to carry out studies of disease susceptibility in more restricted groups is mandatory.

Cryofibrinogenemia triggered by a monoclonal paraprotein successfully treated with cyclophosphamide.

Cryofibrinogenemia is a rare clinical finding with a yet unknown physiopathogenic mechanism. We describe the case of a woman with cold-induced extensive necrotic lesions that responded poorly to initial corticosteroid and anticoagulant therapies. Serum cryoglobulin determinations were persistently negative. After several years of evolution, she developed severe cold-related skin lesions that required left-leg amputation. Further analysis disclosed the presence of cryofibrinogen and an apparently insignificant serum monoclonal immunoglobulin Gκ peak. We additionally demonstrate that the cold precipitation of fibrinogen was directly related to the monoclonal paraprotein presence. The lesions responded dramatically to a B cell-targeted therapy with intravenous cyclophosphamide and dexamethasone.

Decreased circulating endothelial progenitor cells as an early risk factor of subclinical atherosclerosis in systemic lupus erythematosus.

OBJECTIVE: Endothelial progenitor cells (EPCs) play an important role in vascular damage repair and it has been suggested that a decreased number of these cells is associated with increased subclinical atherosclerosis. Our study aim was to evaluate whether the number of circulating EPCs in patients with SLE is associated with subclinical atherosclerosis, the presence of cardiovascular (CV) risk factors and SLE-specific factors. METHODS: Forty-six female SLE patients were included. At the time of each patient's appointment, CV risk factors, SLE-specific factors and EPCs were assessed in peripheral blood by flow cytometry. Simultaneously, atherosclerosis was assessed by measuring the carotid-femoral pulse wave velocity (PWV) by Doppler velocimetry, intima media thickness (IMT) and carotid plaque by B-mode US scanning. RESULTS: Patients were classificated according to PWV following the reference values adjusted by age and blood pressure published by the European Society of Cardiology. Patients with pathological values of PWV showed a significant decrease of circulating EPC percentage compared with normal PWV patients. Decreased EPC counts were also associated with certain risk factors, including hypertension, tobacco use, impaired glucose metabolism, and metabolic syndrome, and correlate with high levels of high-sensitivity CRP (hsCRP) or fibrinogen. The presence of carotid plaque and IMT measurement were unrelated with EPC quantification. CONCLUSION: Patients with a reduced percentage of EPCs showed pathological arterial stiffness and association with certain CV risk factors, suggesting that the measurement of circulating EPCs can be used as a biological marker to determine subclinical atherosclerosis in SLE.

Síndrome de Schnitzler: presentación de un caso y revisión de la respuesta al tratamiento con anakinra / Schnitzler's syndrome: A case report and literature review of the response to treatment with anakinra

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Neurosarcoidosis aislada del nervio óptico / Neurosarcoidosis involving the optic nerve

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Contribution of monocytes to overproduction of interleukin-6 in a case of cardiac myxoma.

Cardiac myxoma cells have been demonstrated to produce interleukin (IL)-6, but its role in the systemic and immunologic manifestations of patients with this tumor is controversial. There is no evidence of any other source of such cytokine, but here we report a case of myxoma with systemic manifestations at diagnosis in which we evaluated the IL-6 production by peripheral blood cells before and after surgical tumor resection. Peripheral blood mononuclear cells were isolated by density gradient centrifugation, and the IL-6 expression in monocytes and lymphocytes was evaluated by flow cytometry. Before surgery, 74.4% of monocytes produced IL-6, but 1 month after surgery, a decrease in both the number of monocytes and the percentage of these cells expressing IL-6 was found along with an improvement of systemic and immunologic manifestations. Here, we demonstrated for the first time that monocytes contribute to the elevated production of IL-6 in patients with myxoma.

Monocytes and T lymphocytes contribute to a predominance of interleukin 6 and interleukin 10 in systemic lupus erythematosus.

OBJECTIVE: To investigate the contribution of T lymphocytes and monocytes to cytokine production in systemic lupus erythematosus (SLE). METHODS: Forty-five SLE patients and 19 healthy volunteers were included. Serum levels of tumor necrosis factor alpha (TNFalpha), interferon gamma (IFN gamma), interleukin (IL)-6, and IL10 were quantified by ELISA. The cytokine production capacities of peripheral blood mononuclear cells were assessed by culturing in vitro with PMA+Ionomycin or LPS. The intracellular cytokine expression was measured by flow cytometry in T lymphocytes and monocytes, respectively. The influence of the disease activity (measured as the SLE-disease activity index; SLEDAI) and the treatment the patients were receiving was evaluated. RESULTS: Serum IL10, IL6, and TNFalpha levels were increased in patients (P

Varón de 35 años con sordera neurosensorial y queratitis intersticial / No disponible

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Common variable immunodeficiency in systemic lupus erythematosus.

OBJECTIVE: The development of common variable immunodeficiency (CVID) or hypogammaglobulinemia in systemic lupus erythematosus (SLE) is rare. The purpose of this article is to provide a detailed review of lupus-associated CVID and to identify clinical characteristics and laboratory features in patients with SLE-associated CVID. METHODS: We describe 2 patients with SLE and CVID and review the cases published in the English literature highlighting both the demographic and the clinical characteristics and the laboratory and therapeutic aspects of this disorder. RESULTS: Detailed descriptions of 18 patients were available; 89% were females with a mean age at the onset of SLE of 23.8 years. In 50% of patients CVID developed within the first 5 years after the diagnosis of SLE. All patients had been treated with corticosteroids and 72% had also received immunosuppressive therapy. Sinopulmonary infections were the most frequent symptom. SLE disease activity decreased after the development of CVID in 67% of patients. Most patients (89%) were treated with gammaglobulin therapy. The most notable immunological feature was a reduced number or percentage of B-cells in 60% of patients. CONCLUSIONS: CVID should be suspected in any SLE patient with recurrent sinopulmonary infections in the absence of SLE activity and/or immunosuppressive treatment.

Bronquiectasias en el lóbulo medio e infección por Mycobacterium avium complex: síndrome de Lady Windermere / Middle lobe bronchiectasis and Mycobacterium avium complex infection: the Lady Windermere Syndrome

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Prognostic value of neutrophils and NK cells in bronchoalveolar lavage of sarcoidosis.

BACKGROUND: Not all the patients with sarcoidosis need pharmacological therapy, and the decision to start therapy is based mainly on clinical conditions. The aim of this study was to evaluate the prognostic value of the leukocyte and lymphocyte subpopulations in the bronchoalveolar lavage fluid from these patients. METHODS: Thirty-three nonsmoking patients with sarcoidosis were included and classified based on the presence of Löfgren's syndrome (n = 11), the radiological stage (12 at Stage I, 17 at Stage II, and 4 at Stage III), and their follow-up. Differential leukocyte subsets and the lymphocyte subpopulations were determined by flow cytometry. RESULTS: The percentage of neutrophils was lower in patients with Löfgren's syndrome (P = 0.038) and in patients at Stage I (P = 0.002). Patients with a poor outcome had a higher percentage of neutrophils (P = 0.004) and NK cells (P = 0.023) than those with a stable disease. Finally, a higher percentage of NK cells was found in those patients who needed a steroid treatment (P = 0.012). CONCLUSIONS: Increased percentages of neutrophils and NK cells in the bronchoalveolar lavage fluid from patients with sarcoidosis are associated with a poor outcome and a higher probability to need steroids treatment. The percentage of neutrophils was also lower in patients with Löfgren's syndrome.

Artritis reumatoide, metotrexato y linfoma no hodgkiniano. Estudio de 3 pacientes / Rheumatoid arthritis, methotrexate and non-Hodgkin’s lymphoma. A report of 3 patients

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Prevalencia de infección por el virus de la hepatitis C en pacientes con linfomas no Hodgkinianos / Prevalence of hepatitis C virus infection in patients with non-Hodgkin's lymphoma

Fundamento: Conocer la prevalencia de la infección por el virus de la hepatitis C (VHC) en pacientes con linfoma no hodgkiniano (LNH-B). Pacientes y método: Estudio transversal de pacientes con LNH-B, comparando serología y ARN del VHC, datos analíticos, toxicidad y respuesta al tratamiento. Resultados: De 77 pacientes, 9 eran positivos para el VHC (11,68 por ciento). El 66 por ciento presentaban elevación de las transaminasas frente al 10,3 por ciento en los pacientes negativos para el VHC. Conclusiones: La prevalencia del VHC es más alta en pacientes con LNH-B que en la población normal. El VHC podría tener un papel en la etiopatogenia de estos linfomas (AU)