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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

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.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.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.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.

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.

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.

El sindrome de Churg-Strauss es una enfermedad multisistemica caracterizada por la presencia de rinitis alergica, asma y eosinofilia periferica1. Los organos mas frecuentemente afectados son el pulmon (90-100%) y la piel (51-70%), junto con la afectacion del sistema nervioso periferico en forma de mononeuritis multiple (66-76%)1,2. Las manifestaciones musculoesqueleticas son posibles durante la fase activa de la enfermedad, siendo mas raras en otros momentos1. La afectacion muscular en forma de miositis en un sindrome de Churg-Strauss es inusual, habiendose publicado solo 3 casos hasta la fecha como forma de presentacion clinica3—5. Presentamos el caso de un paciente con clinica indicativa de polimiositis como primera manifestacion de un sindrome de Churg-Strauss.

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.

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 (TNFα), interferon gamma (IFNγ), 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 TNFα levels were increased in patients (P ≤ 0.01), and a higher spontaneous (without stimuli) intracellular expression of IL10 in CD4+ and CD8+ T lymphocytes (P < 0.05) and of IL6 in monocytes (P = 0.01) were found. After stimulation, patients presented a higher percentage of CD4+ and CD8+ T lymphocytes producing IL4 and IL10 (P ≤ 0.01), and of monocytes producing IL6 (P = 0.04) and IL10 (P = 0.008). The SLEDAI score was positively correlated with the percentage of CD4+IL10+ and CD8+IL10+ T lymphocytes (P < 0.01), and inversely correlated with CD8+TNFα+ (P= 0.02), CD4+IFNγ+ (P = 0.04) and CD8+ IFNγ+ (P = 0.002) T lymphocytes. Patients receiving high dose prednisone produced higher IL10, but they also were the patients with a more active disease. Conclusion Monocytes and T lymphocytes (CD4+ and CD8+) contribute to an overproduction of IL6 and IL10 in SLE; this correlates with the disease activity but is independent of the treatment the patients are receiving. © 2009 Clinical Cytometry Society

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 Lofgren'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 Lofgren'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 Lofgren's syndrome. © 2006 International Society for Analytical Cytology

Current evidence indicates that regulatory T cells (Tregs) actively suppress autoreactive lymphocytes that escape central tolerance.1,2 Tregs are characterised by a high intensity CD25 constitutive expression3 on the surface of CD4 T lymphocytes, which distinguishes them from the non-regulatory CD4+CD25+ T lymphocytes.4 Because of their important role in the maintenance of tolerance, it has been suggested that Tregs are decreased in patients with systemic lupus erythematosus (SLE).5 We have quantified Tregs in patients with SLE and controls and evaluated their association with disease activity and treatment. Thirty three patients with SLE (26 women, 7 men) and 14 healthy volunteers matched for age and sex (11 women, 3 men) with a mean age of 39.9 and 36 years, respectively, were studied. All patients fulfilled at least four of the American College of Rheumatology 1982 revised criteria for the classification of SLE.6 Disease activity at the time of blood sampling was assessed using the SLE Disease Activity Index (SLEDAI).7 …

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.

Visceral leishmaniasis is an infectious disease that occurs only rarely in recipients of solid organ grafts but is associated with an elevated mortality rate despite proper treatment. We report five cases diagnosed in our hospital. All the patients were men aged 30 to 60 years who had undergone kidney transplantation (3 patients), heart transplantation (1), or liver transplantation (1). Three of the patients died, one had multiple recurrences, and one developed post-kala-azar cutaneous leishmaniasis. We review the clinical features, treatments, and outcomes of 26 previously reported cases, pointing out the lower cure rate associated with human immunodeficiency virus infection.

Fundamento Conocer la prevalencia de la infeccion por el virus de la hepatitis C (VHC) en pacientes con linfoma no hodgkiniano(LNH-B) Pacientes y metodo Estudio transversal de pacientes con LNH-B, comparando serologia y ARN del VHC, datos analiticos, toxicidady respuesta al tratamiento Resultados El 66% presentabanelevacion de las transaminasas frenteal 10,3% en los pacientes negativos para el VHC. Conclusiones La prevalencia del VHC esmas alta en pacientes con LNH-B que en lapoblacion normal. El VHC podria tener unpapel en la etiopatogenia de estos linfomas