Abstract Objective To determine the value of whole-body 18 F fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) for diagnosing occult malignant disease in patients with ...myositis compared with broad conventional cancer screening. Methods We prospectively studied 55 consecutive patients with a recent diagnosis of myositis in 3 teaching hospitals over a 3-year period by whole-body FDG-PET/CT and compared the results with those of conventional cancer screening, which included thoracoabdominal CT, mammography, gynecologic examination, ultrasonography, and tumor marker analysis. Comparisons were made using predictive values and their 95% confidence intervals. Results A total of 9 of 55 patients were diagnosed with paraneoplastic myositis. FDG uptake was positive in 7 patients (1 false-positive), negative in 44 patients (3 false-negative), and inconclusive in 4 patients. Positive and negative predictive values of FDG-PET/CT for the diagnosis of cancer were 85.7% and 93.8%, respectively. Conventional screening was cancer-positive in 9 patients (2 false-positive) and negative in the remaining 46 patients (2 false-negative). Positive and negative predictive values were 77.8% and 95.7%, respectively. The overall predictive value of broad conventional screening was the same as that of FDG-PET/CT (92.7 vs 92.7). Conclusion The performance of FDG-PET/CT, a single imaging study, for diagnosing occult malignant disease in patients with myositis was comparable to that of broad conventional screening, which includes multiple tests.
Abstract Background The study of dietary patterns is gaining interest. Although the health benefits of yogurt and lignans have been investigated separately, to our knowledge there are no studies on ...their associative effects. Objective The aim of this study was to evaluate a possible association between yogurt and lignans using biomarkers of cardiovascular disease risk in an elderly population. Design We conducted a cross-sectional analysis of the association between baseline dietary information and cardiovascular risk parameters using food frequency questionnaires. Participants We enrolled 7,169 Spanish participants of the PREDIMED (Prevención con Dieta Mediterránea) study (elderly men and women at high cardiovascular risk) from June 2003 to June 2009. Main outcome measures Cardiovascular risk parameters, including cholesterol, triglycerides, glucose, body mass index, weight, waist circumference, and blood pressure were measured. Statistical analysis General linear models were used to assess the relationship between categorical variables (yogurt, total dairy intake, lignans, and yogurt plus lignans) and cardiovascular risk parameters. Results The consumption of either yogurt or lignans seems to have beneficial effects on human health, but the consumption of both showed greater improvement in some cardiovascular health parameters. Indeed, participants with a higher consumption of both yogurt and lignans showed lower total cholesterol (estimated β-coefficients=−6.18; P =0.001) and low-density lipoprotein cholesterol levels (β=−4.92; P =0.005). In contrast, participants with lower yogurt and lignan consumption had a higher body mass index (β=0.28; P =0.007) and weight (β=1.20; P =0.008). Conclusions High lignan and yogurt consumption is associated with a better cardiovascular risk parameters profile in an elderly Mediterranean population. Further research is warranted to determine the mechanisms and consequences of this potential effect.
Objective To investigate the incidence of clinical and immunological characteristics of a large cohort of Spanish patients with scleroderma (SSc) and identifying factors associated with particular ...organ manifestations assessed by a nationwide cross-sectional analysis. Methods We classified SSc patients in 4 subsets using a modification of LeRoy and Medsger classification that included: “prescleroderma” (pre-SSc), limited cutaneous SSc (lcSSc), diffuse cutaneous SSc (dcSSc), and SSc sine scleroderma (ssSSc). Fourteen Spanish centers participated in patient recruitment. On January 2008, the database included 916 consecutive Spanish SSc patients, 801 women (87.4%) and 115 men (12.6%), all of whom fulfilled the classification criteria proposed by LeRoy and Medsger. Epidemiological, clinical, and laboratory data were collected according to a standard protocol. Mean age at diagnosis was 51.2 ± 15.1 years and mean age at disease onset was 44.9.0 ± 15.8 years. lcSSc was the most frequent subset (61.8%) followed by dcSSc (26.5%), ssSSc (7.5%), and preSSc (4%) subsets. Gender ratios were as follows: dcSSc subset, 200 women and 43 men (4.7:1); lcSSc subset, 503 women and 63 men (ratio 7.9:1), and ssSSc subset, 62 women and 7 men (ratio 8.9:1). Digital ulcers, interstitial lung disease (ILD), musculoeskeletal and esophageal involvement, and scleroderma renal crisis were more frequent in dcSSc than lcSSc and ssSSc subsets. The incidence of pulmonary arterial hypertension assessed by echocardiography was similar in all subsets but mean estimated systolic pulmonary arterial pressure was higher in ssSSc than in lcSSc subset (47.3 ± 23.9 mm Hg vs 39.6 ± 19.2 mm Hg; P < 0.03). Cardiac involvement was identified more frequently in ssSSc than in dcSSc and lcSSc subsets (49.3% vs 32.5% and 31.1%, respectively; P = 0.015 and P = 0.004 for both comparisons). Acro-osteolysis (8.2% vs 2.4%, P = 0.049), calcinosis (19.8% vs 7.2%, P < 0.05), and sicca syndrome (37.5% vs 14.5%, P < 0.0001) were more frequent in lcSSc than in ssSSc subsets. The frequency of clinical manifestations related to the presence of anticentromere antibodies or antitopoisomerase I antibodies was very similar to that identified in patients categorized to lcSSc and dcSSc, respectively. However, in multivariate studies, the ranking of the variables according to their overall explanatory effect on the model showed that the contributory effect of the antibody status was not greater than that of the clinical categorization into lcSSc and dcSSc for the majority of disease manifestations, but, in important manifestations, as ILD, absence of anticentromere antibodies was an independent predictor factor. Conclusions The classification of SSc into dcSSc, lcSSc, and ssSSc subsets is the one that most closely reflects the natural history of the disease, as they presented clear clinical differences. The immunological profile helps to define important visceral alteration as ILD. Finally, to improve early diagnosis of SSc, patients with preSSc should be considered both to trace the true evolution of the disease and to define which patients could benefit from therapeutic measures able to prevent the appearance of visceral involvements.
The objective of the study is to investigate the impact of anemia (defined as hemoglobin concentration of <12 g/dl in women and 13 g/dl in men) on prognosis and to study the effect of recovery from ...anemia on echocardiographic and clinical parameters in patients with aortic stenosis (AS). This was a prospective study in 315 patients with moderate or severe AS. Patients with anemia received oral iron (ferrous sulfate with mucoproteose, 160 mg iron/day) and erythropoietin, if needed, or intravenous iron, if necessary. The following tests were performed before and after normalization of hemoglobin values: echocardiogram, 6-minute walk test, N-terminal B-type natriuretic peptide, and measures of depression, cognitive impairment, and dependence. Patient mean age was 74 years (SD 9). Mean follow-up was 25 months (SD 8). Anemia prevalence in the overall group was 22% (n = 70). Patients who are anemic had a higher rate of complications at follow-up (mortality, hospital admission, or need for valve procedure; 80% vs 62%, p = 0.009). In total, 89% of patients recovered from anemia, with a mean time to recovery of 4.6 weeks (SD 1.4). Improvements were observed on echocardiographic parameters of peak velocity (4.1 to 3.7 m/s, p = 0.02) and mean gradient (44 to 35 mm Hg, p = 0.02). Performance on the 6-minute walk test improved from 235 to 303 m (p <0.001). Median N-terminal B-type natriuretic peptide value decreased from 612 to 189 pg/dl (p <0.001). In conclusion, patients with AS and anemia have a worse prognosis than those without anemia. Resolution of anemia is associated with improvements in echocardiographic parameters and functional status, suggesting that treatment of iron deficiency is a relevant option in the management of patients with AS, particularly in nonoperable cases.
Objective To investigate whether rheumatoid arthritis (RA) and psoriatic arthritis (PsA) can be differentiated in the early stages of the disease (duration of symptoms ≤1 year) on the basis of ...magnetic resonance imaging (MRI) features of the hand and wrist. Material and methods Twenty early RA and 17 early PsA patients with symptomatic involvement of the wrist and hand joints and inconclusive radiographic studies were examined prospectively with contrast-enhanced MRI. Images were evaluated in accordance with the Outcome Measures in Rheumatology Clinical Trials recommendations. Results Certain MRI features, such as the presence of enthesitis or extensive diaphyseal bone marrow edema, were observed exclusively in PsA ( P = 0.0001). These distinctive findings were present in nearly 71% (12/17) of PsA patients. Diffuse and, in some cases, pronounced soft-tissue edema spreading to the subcutis was also seen more frequently in patients with PsA ( P = 0.002). There were no significant differences in the frequency of synovitis, bone erosions, subchondral bone edema, or tenosynovitis between the 2 groups. However, in RA extensor tendons were involved more often than the flexor tendons, whereas in PsA the opposite was observed ( P = 0.014). With respect to the discriminatory power of the different MRI findings examined, only the presence of enthesitis or diaphyseal bone edema and, to a lesser extent, the pattern of hand tendon involvement and the presence of soft-tissue edema accurately differentiated PsA from RA (all these features achieved accuracies greater than 0.70). Conclusions We observed significant differences in the MRI findings of the hand and wrist that can help to distinguish between RA and PsA in the early stages of disease. This imaging method could help to assist in the differential diagnostic process in selected patients in whom diagnosis cannot be unequivocally established after conventional clinical, biochemical, and radiographic examinations.
Objective To review and summarize published information on the effectiveness and safety of rituximab (RTX) in adult patients with refractory neuropsychiatric systemic lupus erythematosus (NPSLE). ...Methods We describe a patient with persistently active NPSLE, despite conventional therapy, who responded dramatically to RTX. Current evidence on the therapeutic use of RTX in this complex situation is also analyzed through a systematic review of the English-language literature, based on a PubMed search. Results Available data on the use of RTX in refractory NPSLE come from a large number of case reports and some open-label studies. Including our case, 35 patients have been well documented. A complete or partial therapeutic response was achieved in 85% of patients after 1 cycle of treatment. A positive correlation between serological markers of disease activity and clinical outcome has also been demonstrated in some of these patients. Clinical improvement was accompanied by a significant reduction in the daily dose of oral corticosteroids. Relapse after RTX treatment was noted in 45% of cases (median 9.5 months; range, 4-33 months). Infections were observed in 29% of patients. Conclusion Evidence for the effectiveness of RTX as induction therapy in NPSLE is based solely on several case reports and noncontrolled trials. Although it is not yet possible to make definite recommendations, the global analysis of these cases supports the off-label use of RTX in cases of severe refractory NPSLE.
Objectives To study the presence and characteristics of nailfold capillary changes in a cohort of adult patients with inflammatory myopathies, determine correlations with disease activity and ...severity, and investigate any relationship between capillary findings and the immunological or clinical characteristics of the groups. Methods Fifty-three consecutive adult patients followed in our outpatient clinic were evaluated using a Wild M3 stereomicroscope with an Intralux 5000 Volpi cold light lamp. A semiquantitative rating scale was used to score capillaroscopy changes. Disease activity and severity were assessed with the Myositis Disease Activity Assessment Tool and Myositis Damage Index, respectively. Associations between capillaroscopy findings and other factors were calculated with the χ2 and Mann–Whitney U tests. Serum autoantibody profile was determined in all patients. Results Twenty-three patients (43%) showed relevant capillaroscopy changes. No significant association was observed between the number of capillaroscopy alterations and the clinical or immunological groups, or disease duration. Disease activity and severity were both significantly associated with a larger number of capillaroscopy findings ( P < 0.05). The combination of microhemorrhages and capillary enlargement was significantly more frequent in patients with dermatomyositis (OR, 8.9; 95% CI, 1.8-45.2), and a characteristic capillaroscopy pattern was associated with paraneoplastic myositis (OR, 14.7; 95% CI, 2.0-106.4). Interstitial lung disease significantly correlated with higher capillary score (OR, 3.7; 95% CI, 1.1-13.0). Conclusions Nailfold microcirculation as determined by semiquantitative simple capillaroscopy appears to provide useful information in patients with idiopathic inflammatory myopathy, contributing to an early diagnosis and identifying patients with a poor prognosis.
Abstract Background and purpose Idiopathic inflammatory myopathies (IIM) are systemic diseases, characterized by the presence of an inflammatory muscle infiltrate. Although more frequent in women, ...its relationship with pregnancy has not been extensively studied. Our goal was to analyze the interaction between pregnancy and myositis in a cohort of IIM women from a single center. Methods A total of 51 patients from a historical cohort of IIM diagnosed between 1983 and 2013 were interviewed with a specific questionnaire. Comparisons between pregnancies occurring before and after the onset of the disease were performed using generalized mixed-effect models with normal and binomial distributions adjusted for confounding factors and clustering. Results A total of 102 pregnancies from 51 patients (41 with dermatomyositis and 10 with polymyositis) were analyzed. A total of 14 pregnancies from 8 patients occurred after disease onset; statistically significant ( p = 0.02) clinical improvement during gestation was evident in 7 pregnancies (4 patients), 5 of them (from 2 patients) experienced a relapse of IIM symptoms afterwards, while in the rest, there was no influence of pregnancy on the disease. No disease flare associated with pregnancy was observed. Two patients were diagnosed within the first 6 months after delivery and none during pregnancy. No evidence was found to support pregnancy as a trigger for myopathy ( p = 0.71). Conclusions Pregnancy does not seem to carry a worse prognosis for the mother nor for the fetus in patients with IIM; on the contrary, nearly half of the patients in our series improved clinically when they became pregnant, a relapse of IIM symptoms being common afterwards. Pregnancy does not appear to be a trigger for IIM.
Preoperative anemia is a risk factor for postoperative morbidity and in-hospital mortality in cardiac surgery. However, it is not known whether treatment of anemia before cardiac surgery by ...administering recombinant human erythropoietin (rhEPO) plus iron improves postoperative outcomes and decreases red blood cell transfusions in these patients. In 1998 a collection of consecutive data for patients who underwent valve replacement was initiated and the inclusion criterion was anemia. Treatment with rhEPO was given at a dose of 500 IU/kg/day every week for 4 weeks and the fifth dose 48 hours before valve replacement. During each rhEPO session, patients received intravenous iron sucrose supplementation. The intervention cohort (2006 to 2011) included 75 patients and the observation cohort was composed of 59 patients who did not receive any treatment (1998 to 2005). Multivariable logistic regression analysis showed that administration of combined therapy was independently associated with decreased postoperative morbidity (odds ratio OR 0.13, 95% confidence interval CI 0.03 to 0.59 p = 0.008) and in-hospital mortality (OR 0.16, 95% CI 0.28 to 0.95 p = 0.04) after adjusting for logistic European System for Cardiac Operative Risk Evaluation score, type of intervention, time of cardiopulmonary bypass, and year of surgery. Individually, this treatment also decreased postoperative renal failure (OR 0.23, 95% CI 0.06 to 0.88, p = 0.03). Rate of red blood cell transfusion decreased from 93% in the observation cohort to 67% in the intervention cohort as did days of hospitalization (median, 15 days, 10 to 27, versus 10 days, 8 to 14, respectively, p = 0.01 for all comparisons). In conclusion, administration of intravenous rhEPO plus iron in anemic patients before valve replacement improves postoperative survival, decreases blood transfusions, and shortens hospitalization.
Objective To review and summarize the information available on the effectiveness and safety of biological therapies in refractory Felty's syndrome (FS). Methods We describe a case of FS with severe ...neutropenia and recurrent bacterial infections unresponsive to disease-modifying antirheumatic drug treatment and long-term administration with granulocyte colony-stimulating factor, in which treatment with rituximab (RTX) was useful and resulted in a sustained neutrophil response. Current evidence on the use of biological therapies in FS is also analyzed through a systematic review of the English-language literature, based on a PubMed search. Results Available data on the use of biological therapies in refractory FS are based only on several case reports and are limited to the use of RTX and some anti-tumor necrosis factor α agents (etanercept, infliximab, and adalimumab). Including the case described here, data are available on 8 patients treated with RTX. A sustained increase in the absolute neutrophil count (>1500/mm3 ) was observed in 62.5% (5/8) of these patients after 1 cycle of treatment. In most of them, the hematological response was accompanied by a parallel improvement in biological markers of inflammation and other clinical manifestations of FS (arthritis, recurrent infections, systemic symptoms, etc). After a median follow-up of 9 months (range, 6-14), only 1 of these patients relapsed and neutropenia reappeared; in this patient, retreatment was rapidly effective. No significant adverse events related to RTX therapy were reported. Experience with anti-tumor necrosis factor agents is limited to 6 patients, none of whom presented any sustained increase in neutrophil count. Conclusions Although it is not yet possible to make definite recommendations, the global analysis of all cases reported to date only supports the use of RTX as a second-line therapy in patients with refractory FS.
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