Evolution of clinical, histological and serological features in a Primary Sjὄgren´s Syndrome cohort and the limitations of the current classification criteria.

OBJECTIVE: The classification and/or diagnosis of Primary Sjögren's Syndrome (PSS) requires a multidimensional approach. Although age and the duration of sicca symptoms can affect the clinical, serological and histological features found at initial evaluation, these are not considered when using classification criteria as a guide for PSS diagnosis. Our study aimed to explore if there is any relationship between the duration of symptoms and clinical, histopathological and serological findings. METHODS: An observational, retrospective study was performed. All the evaluated subjects were part of the "sicca cohort". Patients' clinical, serological and histological characteristics were assessed according to the duration of symptoms. A Receiving Operator Characteristic (ROC) curve was performed to establish the duration of symptoms (months) that predicted a PSS diagnosis. Binary regression models and odds ratios were used to evaluate the association between the duration of symptoms and the clinical, serological, and histopathological profiles. RESULTS: One hundred and sixteen patients were included; 97(83.62%) fulfilled PSS criteria. Of the 116 patients, thirty-six (31.03%) had < 15 months presenting with sicca symptoms when receiving a diagnostic approach. A duration of symptoms >15 months was associated with an altered Schirmer test (OR 2.76; 95% CI 1.15-6.61, P=0.02), low salivary flow rate (OR 3.5; 95% CI 1.34-9.13, P=0.01), ≥1 foci score (OR 1.21; 95% CI 1-1.45, P=0.04), ocular (OR 7.8; 95% CI 1.49-40.81, P=0.02) and severe oral symptoms (OR 2.61; 95% CI 1.16-5.87, P=0.02). CONCLUSION: The time of evolution of symptoms plays a fundamental role in the clinical, histological and serological profiles in PSS.

Knowledge and attitudes about influenza vaccination in rheumatic diseases patients.

Patients with rheumatic diseases (RD) have a higher risk of morbidity and mortality from vaccine-preventable infections attributed to disease activity, comorbidities, immunosuppressive therapy, and other factors. Vaccines are one of the safest and most effective public health interventions. The aim of this study was to investigate knowledge and attitudes about influenza vaccination as factors influencing vaccine uptake and hesitancy in a population with RD. A descriptive cross-sectional study was designed. A self-administered questionnaire surveyed age, RD diagnosis, ten questions about the uptake, safety and efficacy of influenza vaccine, knowledge of cost-free availability, and the relationship between influenza vaccination and RD. A total of 223 questionnaires were filled; 79.8% of patients were vaccinated for influenza at least once. Uptake by diagnosis was 80.3% in rheumatoid arthritis, 76.2% in osteoarthritis, 86.7% in lupus, 73.9% in other auto-immune diseases (AID), and 60% in other non-AID; 83.9% of patients considered influenza vaccine as safe and effective. From those who had never been vaccinated, 26.7% of patients did not consider influenza vaccine safe and effective vs. 13.5% among patients who had been vaccinated (P = .032). Only 7.6% considered that RD patients could not be vaccinated; 11.7% thought that influenza vaccine would worsen their RD symptoms. This study showed that concerns about safety, efficacy, side effects, fear of the vaccine, and knowledge of cost diminished vaccine uptake. These are factors related to confidence, complacency, and convenience as components of vaccine hesitancy that affect influenza vaccination in RD patients.

Vaccination in rheumatic disease patients: results of a pilot quality improvement program.

OBJECTIVES: Patients with rheumatic diseases (RD) are at increased risk of infections. Vaccination is recognized as a successful public health measure and is recommended for RD patients. The aim of this study was to describe the strategies implemented in an academic rheumatology outpatient clinic as part of a fellow-in-training-led vaccination quality improvement (QI) program and to explore the vaccination uptake before and one year after the implementation. MATERIAL AND METHODS: The program's objective is the promotion of vaccination among patients and rheumatology fellows (by educational interventions, development of vaccination charts and orders, and modifications to electronic medical records to register vaccination dates and generate reminders). As part of the continuous evaluation of the QI program, a descriptive cross-sectional study was performed to evaluate vaccine uptake pre- and post-interventions and vaccination barriers one year after implementation. Consecutive patients with RD answered a self-administered questionnaire. Results are shown as descriptive statistics. RESULTS: Before the program started 73 patients were surveyed and 102 patients one year after. The vaccination uptake rates for influenza pre- and post-interventions were 43% and 55%; for pneumococcal vaccination they were 26% and 30%; for herpes zoster they were 0% and 4%; for human papillomavirus they were 4% and 10%; for hepatitis B (HBV) they were 19% and 25% respectively. Eighty percent of patients reported some barriers to receiving any of the previous vaccines. The three main reasons for not receiving a vaccine were the lack of recommendation, the lack of availability, and the belief that vaccines do not work. CONCLUSIONS: The implementation of a pilot vaccination QI program led by rheumatology fellows-in-training showed promising preliminary benefits in the vaccination uptake among RD patients and helps to evaluate the barriers to surpass.

High frequency of primary refractory disease and low progression-free survival rate of Hodgkin's lymphoma: a decade of experience in a Latin American center

Abstract Background: Reports dealing with clinical outcomes of classical Hodgkin's lymphoma in low- to middle-income countries are scarce and response to therapy is poorly documented. This report describes the characteristics and clinical outcomes of patients with classical Hodgkin's lymphoma from a single institution in Latin America. Method: A retrospective study was conducted over ten years of patients with classical Hodgkin's lymphoma treated at a referral center. Progression-free and overall survival rates were estimated by Kaplan-Meier analysis. The univariate Cox regression model was used to estimate associations between important variables and clinical outcomes. Main results: One hundred and twenty-eight patients were analyzed. The mean age was 28.5 years. The five-year progression-free and overall survival were 37.3% and 78.9%, respectively. Of the whole group, 55 (43%) were primary refractory cases. Only 39/83 (47%) patients with advanced disease vs. 34/45 (75.6%) in early stages (p-value = 0.002) achieved complete remission. Those with advanced disease had a five-year overall survival of 68.7% vs. 91.8% for early disease (p-value = 0.132). Thirty-one patients relapsed (24.2%) and 20 (64.5%) received a transplant. The hazard ratio for progression with bone marrow infiltration was 2.628 (p-value = 0.037). For death, an International Prognostic Score ≥4 had a hazard ratio of 3.355 (p-value = 0.050) in univariate analysis. Two-thirds of classical Hodgkin's lymphoma patients diagnosed at advanced stages had a low progression-free survival but an overall survival similar to high-income countries. Conclusion: Patients diagnosed with classical Hodgkin's lymphoma in Northeastern Mexico had a significantly low progression-free survival rate and presented with advanced disease, underscoring the need for earlier diagnosis and improved contemporary therapeutic strategies in these mainly young productive-age Hodgkin's lymphoma patients.

High frequency of primary refractory disease and low progression-free survival rate of Hodgkin's lymphoma: a decade of experience in a Latin American center.

BACKGROUND: Reports dealing with clinical outcomes of classical Hodgkin's lymphoma in low- to middle-income countries are scarce and response to therapy is poorly documented. This report describes the characteristics and clinical outcomes of patients with classical Hodgkin's lymphoma from a single institution in Latin America. METHOD: A retrospective study was conducted over ten years of patients with classical Hodgkin's lymphoma treated at a referral center. Progression-free and overall survival rates were estimated by Kaplan-Meier analysis. The univariate Cox regression model was used to estimate associations between important variables and clinical outcomes. MAIN RESULTS: One hundred and twenty-eight patients were analyzed. The mean age was 28.5 years. The five-year progression-free and overall survival were 37.3% and 78.9%, respectively. Of the whole group, 55 (43%) were primary refractory cases. Only 39/83 (47%) patients with advanced disease vs. 34/45 (75.6%) in early stages (p-value=0.002) achieved complete remission. Those with advanced disease had a five-year overall survival of 68.7% vs. 91.8% for early disease (p-value=0.132). Thirty-one patients relapsed (24.2%) and 20 (64.5%) received a transplant. The hazard ratio for progression with bone marrow infiltration was 2.628 (p-value=0.037). For death, an International Prognostic Score ≥4 had a hazard ratio of 3.355 (p-value=0.050) in univariate analysis. Two-thirds of classical Hodgkin's lymphoma patients diagnosed at advanced stages had a low progression-free survival but an overall survival similar to high-income countries. CONCLUSION: Patients diagnosed with classical Hodgkin's lymphoma in Northeastern Mexico had a significantly low progression-free survival rate and presented with advanced disease, underscoring the need for earlier diagnosis and improved contemporary therapeutic strategies in these mainly young productive-age Hodgkin's lymphoma patients.

TNF-α increases in the CSF of children with acute lymphoblastic leukemia before CNS relapse.

There is scarce information regarding the concentration of cytokines in cerebrospinal fluid (CSF) of children with acute lymphoblastic leukemia (ALL) and their clinical association with CNS status. A prospective analysis of 40 patients <18years with newly diagnosed ALL was performed. Human cytokine magnetic bead panel assay values of IL-2, IL-4, IL-6, IL-8, IL-10, MCP-1, TNF-α in CSF at diagnosis, end of induction to remission, and 6months after diagnosis were determined. IL-6 and MCP-1 values showed a significant increment at the end of induction. From the whole group 4 (10.0%), patients relapsed to the CNS at a median of 11.48months. A significantly higher value of TNF-α at third determination in these CNS-relapsed patients was documented, 7.48 vs. 2.86pg/mL in 36 children without relapse (p=0.024). TNF-α concentration increased at a median 5.48months before CNS relapse. By receiver-operating characteristic curve (ROC) analysis, the best cut-off point of TNF-α concentration that better predicted CNS relapse was ≥1.79pg/mL. In conclusion an increase in TNF-α concentration on CSF preceded CNS relapse in children with ALL. An increase in MCP-1 and IL-6 was not associated to CNS relapse and appears to result from an inflammatory response after IT injection of chemotherapy.

Non-Hodgkin lymphomas: impact of rituximab on overall survival of patients with diffuse large B-cell and follicular lymphoma.

BACKGROUND AND AIMS: Information comparing treatment outcome for diffuse large B cell (DLBCL) and follicular (FL) non-Hodgkin lymphoma (NHL) patients treated with schemes with and without rituximab from low- and middle-income countries is scarce. Clinical characteristics, response to therapy and long-term outcome of DLBCL and FL patients were studied. METHODS: Patients with DLBCL and FL diagnosed over 8 years at a reference center in northeast Mexico were included. Kaplan-Meier method was used to determine overall survival (OS) and progression-free survival (PFS). Cox regression model was used to evaluate the association between risk factors, rituximab therapy and clinical outcome. RESULTS: One hundred-sixteen patients with DLBCL and 65 with FL in advanced stages were included. Median age was 57.8 and 56 years, respectively. Clinical characteristics between groups receiving or not receiving rituximab were comparable. Stages III and IV were found in 63.8% of DLBCL and 84.6% in FL patients, respectively. OS and PFS at 60 months were 63.8 and 51.2% in DLBCL and 70.6 and 33.8% in FL. No difference in OS was found in DLBCL and FL when rituximab-based regimens vs. non rituximab-based regimens were compared, but a statistically significant difference was documented in PFS in FL patients. CONCLUSION: Addition of rituximab to CHOP-like regimens did not improve OS in DLBCL and FL NHL subtypes. In comparison to developed countries, diagnosis of NHL was made a decade earlier and in advanced clinical stages. Cost-efficiency of adding rituximab to therapy for these patients should be assessed.