Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies.

OBJECTIVE: To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. METHODS: By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. RESULTS: There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). CONCLUSION: In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.

Impact of the COVID-19 Pandemic on Primary Health Care Disease Incidence Rates: 2017 to 2020.

We assessed the impact of the COVID-19 pandemic in Spain on new cases of diseases and conditions commonly seen in primary care. In 2020, there were significant reductions from 2017-2019 in the annual incidences of hypertension (40% reduction), hypercholesterolemia (36%), type 2 diabetes (39%), chronic kidney disease (43%), ischemic heart disease (48%), benign prostatic hypertrophy (38%), osteoporosis (40%), hypothyroidism (46%), chronic obstructive pulmonary disease (50%), alcohol use disorder (46%), benign colon polyps and tumors (42%), and melanomas (45%). Prioritization of COVID-19 care changed the physician-patient relationship to the detriment of face-to-face scheduled visits for chronic disease detection and monitoring, which fell by almost 41%. To return to prepandemic levels of diagnosis and management of chronic diseases, primary health care services should reorganize and carry out specific actions for groups at higher risk.VISUAL ABSTRACTAnnals "Online First" article.

Safety and efficacy of SARS-CoV-2 vaccination in 1237 patients with primary Sjögren syndrome.

OBJECTIVES: To investigate the safety and efficacy of SARS-Cov-2 vaccination in patients with primary Sjögren syndrome (pSS) due to scarcity of data in this population. METHODS: By the first week of May 2021, all Big Data SS Consortium centres patients who had received at least one dose of any SARS-CoV-2 vaccine were included in the study. The in-charge physician asked patients about local and systemic reactogenicity to collect SARS-CoV-2 vaccination data. RESULTS: The vaccination data of 1237 patients were received. A total of 835 patients (67%) reported any adverse events (AEs), including local (53%) and systemic (50%) AEs. Subjective symptoms (63%) were the most common local AEs, followed by objective signs at the injection site (16%), and general symptoms were the most commonly reported systemic AEs (46%), followed by musculoskeletal (25%), gastrointestinal (9%), cardiopulmonary (3%), and neurological (2%). In addition, 141 (11%) patients reported a significant worsening/exacerbation of their pre-vaccination sicca symptoms and fifteen (1.2%) patients reported active involvement in the glandular (n=7), articular (n=7), cutaneous (n=6), pulmonary (n=2), and peripheral nervous system (n=1) domains due to post-vaccination SS flares. In terms of vaccination efficacy, breakthrough SARS-CoV-2 infection was confirmed after vaccination in three (0.24 %) patients, and positive anti-SARS-Cov-2 antibodies were detected in approximately 95% of vaccinated SS patients, according to data available. CONCLUSIONS: Our data suggest that patients with pSS develop adequate humoral response and no severe AEs after SARS-CoV-2 vaccination and therefore raise no concerns about the vaccine's efficacy or safety profile in this population.

Long-Term Effects on Preventing Frailty and Health Care Costs Associated with a Multifactorial Intervention in the Elderly: Three-Year Follow-Up Data from the Pre-Frail 80 Study.

INTRODUCTION: Preventing or delaying frailty has important benefits. Studies show the effectiveness of multifactorial interventions in the frail and pre-frail elderly, but few have evaluated their long-term effectiveness. Frailty and its consequences have been shown to increase the use of health resources. The main aim was to evaluate the long-term effect of a multifactorial primary healthcare intervention in pre-frail elderly people at 36 months and determine the health resources used and their cost. METHODS: A follow-up of a cohort study of patients who participated in a randomized clinical trial in an urban primary care centre in Barcelona was carried out. We included 200 non-institutionalized people aged ≥80 years who met the Fried pre-frailty criteria. The intervention group (IG) received a 6-month interdisciplinary intervention based on physical exercise, Mediterranean diet advice, assessment of inadequate prescribing in polypharmacy patients, and social assessment, while the control group (CG) received standard of care primary healthcare treatment. Sociodemographic variables were collected at baseline. The Fried criteria, comorbidities, and geriatric syndromes were collected at baseline and 12 and 36 months. For the analysis of health costs, data were collected on visits, complementary tests, hospital admissions, and surgical interventions in the last 36 months. Complexity, the rate of expected emergency admission, and the rate of expected mortality were collected at 36 months. Between-group characteristics were compared at baseline and 36 months using the χ2 test and the t test for independent samples. The post-intervention (12-month follow-up) versus longitudinal follow-up (36-month follow-up) comparison used McNemar's test for each group. The nonparametric Mann-Whitney test was used to compare health costs. RESULTS: Of the 200 patients initially included, we evaluated 135 (67.5%) patients who completed the 36-month follow-up. The mean age was 88.5 years and 64.4% were female. At 36 months, the transition to frailty was much lower in the IG than in the CG (22.1% vs. 32.8%, p = 0.013). The total mean health cost at 36 months was 3,110 EUR in the CG and 2,679 EUR in the IG. No significant between-group differences were observed according to Clinical Risk Groups. CONCLUSIONS: A multifactorial, interdisciplinary intervention carried out in primary care prevented frailty in pre-frail elderly people at 36-month follow-up. Although the IG was grouped into higher grade Clinical Risk Groups and therefore had greater morbidity, the cost was lower than that in the CG.

SARS-CoV-2 infection in patients with primary Sjögren syndrome: characterization and outcomes of 51 patients.

OBJECTIVE: To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. METHODS: We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. RESULTS: A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. CONCLUSION: Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities.

Validity and reliability of the Spanish caregiver reaction assessment scale for caregivers of people with dementia.

AIM: Of the few instruments available to measure the important, positive aspects of caring, the Caregiver Reaction Assessment is regarded as the most suitable, but there is no validated Spanish version. The aim of this study was to translate the Caregiver Reaction Assessment into Spanish and assess its psychometric properties. METHODS: The Caregiver Reaction Assessment was translated into Spanish and then back translated. Validation included construct validity, criterion validity and reliability: 287 interviews were conducted with informal caregivers of patients with dementia between November 2010 and April 2012. RESULTS: Principal component analysis confirmed the original instrument's five subscales. Criterion validity showed a moderate negative correlation between the impact on health and the EQ-5D (r = -.43), that is, a greater impact on health correlated with lower health-related quality of life. The Caregiver Reaction Assessment showed good internal consistency, with a Cronbach alpha of .804, and good temporal stability for the distinct subscales, with intraclass correlation coefficients varying from .683 to .729 (p < .001). CONCLUSION: The Caregiver Reaction Assessment is a reliable, valid instrument for the measurement of the reactions of informal caregivers of patients with dementia, with good psychometric properties.

Intervention to reduce perceived loneliness in community-dwelling older people.

AIM: We evaluated the reduction in perceived loneliness and depression and the increase in social support and quality of life in community-dwelling lonely people aged >65 years included in a community intervention compared with nonlonely controls from the same urban area. DESIGN: Randomised clinical trial without blind evaluation. LOCATION: Urban area of Barcelona. PARTICIPANTS: Community dwellers aged >65 years with loneliness identified by the primary care team. INTERVENTIONS: The primary care team together with community agents (municipal social services, community civil and religious associations) carried out 18 sessions developing activities including educational workshops, mindfulness, yoga, walking and visits to urban gardens. RESULTS: We included 55 patients (87% female, mean age 80.6 ± 6.86 years) of whom 82% had moderate and 18% severe loneliness. Six months postintervention, 48.3% of the intervention group did not feel lonely compared with 26.9% of controls (p = 0.001). Social support (DUKE-UNC-11) increased from 33.5 ± 9.3 to 41.4 ± 6.6, and mental health (SF-12) from 36 ± 610.4 to 48 ± 11.1 and depressive symptoms (Yesavage test) decreased from 9.2 ± 3.6 to 5.2 ± 5.0 in the intervention but not the control group. CONCLUSIONS: The intervention mainly reached people with moderate loneliness and significantly improved the perception of loneliness, depressive symptoms, social support and the mental health component of the quality of life. The intervention may be more suitable for people with moderate loneliness, but these types of activities may be difficult to accept by people with severe loneliness not related to the barriers to socialisation generated by ageing.

EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies.

The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed at developing the first EULAR evidence and consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. The TF endorsed the presentation of general principles for the management of patients with SjS as three overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence, starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic disease. The recommendations address the use of topical oral (saliva substitutes) and ocular (artificial tear drops, topical non-steroidal anti-inflammatory drugs, topical corticosteroids, topical CyA, serum tear drops) therapies, oral muscarinic agonists (pilocarpine, cevimeline), hydroxychloroquine, oral glucocorticoids, synthetic immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide and mycophenolate), and biological therapies (rituximab, abatacept and belimumab). For each recommendation, levels of evidence (mostly modest) and TF agreement (mostly very high) are provided. The 2019 EULAR recommendations are based on the evidence collected in the last 16 years in the management of primary 2002 SjS patients and on discussions between a large and broadly international TF. The recommendations synthesise current thinking on SjS treatment in a set of overarching principles and recommendations. We hope that the current recommendations will be broadly applied in clinical practice and/or serve as a template for national societies to develop local recommendations.

Autoimmune congenital heart block and primary Sjögren's syndrome: characterisation and outcomes of 49 cases.

OBJECTIVES: To characterise autoimmune congenital heart block (CHB) associated with a maternal diagnosis of primary Sjögren's syndrome (pSS) confirmed either before, concomitant or after the first pregnancy complicated with CHB. METHODS: The following inclusion criteria were applied: (i) Mothers with positive Ro/La autoantibodies detected previously or at the time of diagnosis of the first case of CHB; (ii) diagnosis of CHB confirmed by fetal echocardiography; (iii) AV block diagnosed in uterus, at birth or within the neonatal period (0-27 days after birth) (8); (iv) absence of anatomical cardiac abnormalities which might be causal of AV block; and (v) maternal fulfillment of the 2002 SS criteria before, during or after having a pregnancy complicated with CHB. RESULTS: We identified 49 cases of autoimmune CHB in children born from 44 mothers who had a mean age at the time of pregnancy of 30.3 years (range 18 to 41). At the time of diagnosis of autoimmune CHB, all mothers had positive anti-Ro antibodies and 28/44 (64%) were positive for anti-La antibodies. Only 10 (22%) mothers with affected pregnancies had a diagnosis of primary SS at the time of diagnosis of the first pregnancy complicated by CHB (a mean of 4 years before, ranging from 1 to 10 years). In 6 (14%) mothers, primary SS was diagnosed during pregnancy or less than 12 months after the delivery/termination. In the remaining 28 (64%) mothers, pSS was confirmed 1-5 years after CHB diagnosis (n=19, 68%), 6-10 years after (n=2, 7%), or more than 10 years after the first case of CHB was diagnosed (n=7, 25%). CHB was diagnosed in uterus in all cases but two. AV block was initially incomplete in 11 fetuses and complete in 36 (no available data in 2 cases). Among the 35 (71%) surviving children with CHB, 5 (14%) developed other features of neonatal lupus. After the index pregnancy, 12 women had 20 subsequent pregnancies: five were complicated by a CHB (recurrence rate of CHB of 25%). The 4 women who had recurrent CHB were double-positive for anti-Ro and anti-La antibodies, and all had a confirmed pSS before having the first index case of CHB. CONCLUSIONS: In pSS, autoimmune CHB could be one of the first "indirect" signs of the disease in women of childbearing-age, in whom the diagnosis is confirmed several years later. Some maternal characteristics could be related with recurrent CHB, such as having an already-confirmed diagnosis of pSS and carrying the two Ro/La autoantibodies.

Spanish version of the Satisfaction with Epilepsy Care questionnaire: Adaptation and psychometric properties.

OBJECTIVE: The objective of this study was to perform a cross-cultural adaption and psychometric evaluation of the Spanish version of the Satisfaction with Epilepsy Care (SEC) questionnaire and analyze patient satisfaction with epilepsy care. METHODS: Transcultural adaptation and validation of the SEC were carried out using translation and back-translation with pilot testing and an expert panel. The SEC-E (Spanish) was analyzed in 213 patients with epilepsy to examine construct and criterion validity and internal consistency. RESULTS: The SEC-E achieved conceptual, semantic, and content equivalence with the original version. For content validity, one question was eliminated from the original questionnaire as it has little relevance in our cultural setting. Positive correlations for criterion validity were obtained using the gold standard measure (Satisfaction in Hospitalized Patients scale). Construct validity replicated the three dimensions of the original questionnaire. The scale showed adequate reliability through internal consistency (Cronbach's α of 0.94) and temporal stability on retest (n = 85). Patients scored (0 to 100) 77.5 [standard deviation (SD): 19.9] for satisfaction with communication, 76.9 (SD: 17) for organization, and 67.2 (SD: 22.1) for information. SIGNIFICANCE: The SEC-E is a valid and reliable tool for the assessment of educational interventions aiming to improve the quality of care in patients with epilepsy in Spanish clinical practice. The results showed a good level of patient satisfaction with epilepsy care.

Indicator condition-guided HIV testing with an electronic prompt in primary healthcare: a before and after evaluation of an intervention.

OBJECTIVE: Indicator condition (IC)-guided HIV testing is a strategy for the diagnosis of patients with HIV. The aim of this study was to assess the impact on the proportion of HIV tests requested after the introduction of an electronic prompt instructing primary healthcare (PHC) physicians to request an HIV test when diagnosing predefined IC. METHODS: A prospective interventional study was conducted in 2015 in three PHC centres in Barcelona to assess the number of HIV test requests made during the implementation of an electronic prompt. Patients aged 18-65 years without HIV infection and with a new diagnosis of predefined IC were included. The results were compared with preprompt (2013) and postprompt data (2016). RESULTS: During the prompt period, 832 patients presented an IC (median age 41.6 years [IQR 30-54], 48.2% female). HIV tests were requested in 296 individuals (35, 6%) and blood tests made in 238. Four HIV infections were diagnosed (positivity rate 1.7%, 95% CI 0.5% to 4.4%). The number of HIV tests requested based on IC increased from 12.6% in 2013 to 35.6% in 2015 (p<0.001) and fell to 17.9% after removal of the prompt in 2016 (p<0.001). Younger patient age (OR 0.97, 95% CI 0.96 to 0.98), birth outside Spain (OR 1.53, 95% CI 1.06 to 2.21) and younger physician age (OR 0.97, 95% CI 0.96 to 0.99) were independent predictive factors for an HIV test request during the prompt period. The electronic prompt (OR 3.36, 95% CI 2.70 to 4.18) was the factor most closely associated with HIV test requests. It was estimated that 10 (95% CI 3.0 to 26.2) additional new cases would have been diagnosed if an HIV test had been performed in all patients presenting an IC. CONCLUSIONS: A significant increase in HIV test requests was observed during the implementation of the electronic prompt. The results suggest that this strategy could be useful in increasing IC-guided HIV testing in PHC centres.

Geoepidemiological big data approach to sarcoidosis: geographical and ethnic determinants.

In sarcoidosis, a rare multiorgan disease of unknown aetiology characterised by non-caseating epitheloid cell granulomas, three geoepidemiological factors are major aetiopathogenic factors: geolocation, ethnicity, and personal environment. Geographically, sarcoidosis is mainly reported in the Northern Hemisphere, with the highest incidence rates uniformly reported in countries located at the highest latitudes. The main geoepidemiological-driven differences across the world are of greater female involvement in Southern Europe, the Southern US and Japan, a differentiated radiological pattern (predominance of stage I in Southern Europe and Middle East/Asia and of stage II in Northern Europe, China and India, with the US and Japan having the highest frequencies of stages III/IV) and the extrathoracic phenotype: the most frequent extrathoracic organs involved are the skin in Southern Europe and Middle East/Asia, the eyes in Northern Europe, Northeast US and Japan, the liver in India and the lymph nodes in China. In addition, there are large ethnicity-driven variations in the frequency, epidemiology, clinical expression and outcome of sarcoidosis. The highest incidence rates are uniformly reported in Black/African-American people, independently of the geographical location, with rates between 2- and 10-fold higher than those reported in White people living in the same geographical area. Furthermore, ethnicity heavily influences the clinical phenotype by modifying the age at diagnosis and the rates of thoracic and extrathoracic involvements. Geoepidemiological studies enhanced by big data may yield important clues to understanding the role of these factors in the frequency and clinical phenotypes of sarcoidosis.

How the Frequency and Phenotype of Sarcoidosis is Driven by Environmental Determinants.

BACKGROUND: Sarcoidosis is a systemic disease in which the personal environment seems to drive a differentiated disease frequency and clinical expression. The main epidemiological studies suggest a key influence of potential environmentally linked exposures related to the type of occupation, the household, life style, socioeconomic status, and region of residence. OBJECTIVE: To provide an update on how sarcoidosis may be modulated by environmental factors. DATA SOURCES: We searched PubMed for epidemiological studies. SYNTHESIS: The risk of sarcoidosis is enhanced in people working in jobs related to agriculture, water, construction, metal machining, education, and health, and reduced in those working in jobs mainly centered on personal care. Studies have confirmed seasonal-related peaks of sarcoidosis incidence that follow geographical North-South and West-East gradients. Other personal factors include smoking, personal household exposures, and leisure activities. The evidence pointing to the crucial role of the environment in the etiopathogenesis of sarcoidosis is mounting rapidly. Few diseases so strongly combine geography, environment, gender, and ethnicity as key etiopathogenic factors, with susceptibility to any putative agent being modulated by the individual exposome and genome. CONCLUSION: Geoepidemiological research should focus on evaluating the combined effects of environmental and genetic factors, the identification of clusters of geographically driven exposures, and more precise measurement of all personal exposures (degree of combination, length, and level of exposure).

Severe, life-threatening phenotype of primary Sjögren's syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry).

OBJECTIVES: To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS). METHODS: The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain. RESULTS: Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reached to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved. CONCLUSIONS: 13% of patients with primary SS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.

The Burden of Comorbidity and Complexity in Sarcoidosis: Impact of Associated Chronic Diseases.

PURPOSE: To evaluate comorbidity, complexity and poor outcomes in patients with sarcoidosis and to compare those scores with a control group. METHODS: 218 consecutive patients were diagnosed with sarcoidosis according to the ATS/ERS/WASOG criteria; extrathoracic involvement was evaluated using the 2014 WASOG organ assessment instrument. Sarcoidosis patients were compared with an age- and gender-matched control group of primary care outpatients without sarcoidosis. Comorbidities were assessed retrospectively using the Charlson Comorbidity Index (CCI); complexity was evaluated according to the classification into Clinical Risk Groups (CRG) and severity levels. RESULTS: The cohort included 142 women and 76 men; the mean age was 47.1 years at diagnosis of sarcoidosis and 55.9 years at the last visit. Patients with a CCI > 1 had a higher frequency of calcium/vitamin D abnormalities (p < 0.001), kidney involvement (p = 0.005) and a higher mortality rate (p < 0.001) compared with patients with a CCI ≤ 1. Patients with a CRG ≥ 6 had a higher frequency of extrathoracic involvement (p = 0.039), calcium/vitamin D abnormalities (p = 0.019) and treatment with glucocorticoids (p = 0.032) compared with patients with a CRG < 6. 11% patients died after a mean follow-up of 102.3 months. Country of birth, kidney involvement and extrathoracic disease were significantly associated with death. Patients with sarcoidosis had a higher frequency of liver (p < 0.001), pulmonary (p = 0.002) and autoimmune disease (p = 0.011) and cancer (p = 0.007) compared with the control group. CONCLUSION: We found higher rates of comorbidity and complexity in patients with sarcoidosis compared with a control group. Liver, pulmonary, autoimmune and neoplastic diseases were the main comorbidities found in patients with sarcoidosis.

[Effectiveness of an educational program for respiratory rehabilitation of Chronic Obstructive Pulmonary Disease patients in Primary Care in improving the quality of life, symptoms, and clinical risk]. / Efectividad de un programa educativo de rehabilitación respiratoria en atención primaria para mejorar la calidad de vida, la sintomatología y el riesgo clínico de los pacientes con enfermedad pulmonar obstructiva crónica.

AIM: To determine the impact of an educational program to improve the management of chronic obstructive pulmonary disease (COPD) that contributes to an increase of the quality of life, exercise capacity, level of dyspnoea, and clinical risk. DESIGN: Intervention study without controls. LOCATION: Primary Healthcare Centre. PARTICIPANTS: 193 patients with COPD were invited, 73 accepted and 55 participated in the educational program. INTERVENTIONS: Respiratory rehabilitation educational program with basic concepts of pulmonary and respiratory pathophysiology, respiratory physiotherapy exercises, practical workshop on the use of the most frequent inhalation devices, understanding of chronic disease and self-care measures in case of exacerbation. MAIN MEASUREMENTS: The quality of life (the COPD assessment test), exercise tolerance (the Six-Minute Walk Test), rating of perceived exertion (Borg Dyspnoea Score) and clinical risk (BODE index) were assessed by means of validated questionnaires in Spanish. RESULTS: A total of 43 (78.2%) participants completed the program. An improvement in the quality of life by a mean of 3.3 points was observed (95%CI; 1.76-4.84). Just over half (53.5%) of the participants obtained a clinically relevant improvement. Participants also improved their physical exercise capacity at post-intervention by increasing the distance that they walked in 6min by a mean of 20.76m (95%CI; 2.57-38.95). Improvements in the level of dyspnoea and clinical risk were also observed. CONCLUSIONS: The educational program shows a statistically significant and clinically relevant improvement in the quality of life, fatigue, symptomatology, exercise capacity, level of dyspnoea, and clinical risk. The program is adaptable to the health care routine of healthcare centres.

Randomized trial to reduce emergency visits or hospital admissions using telephone coaching to complex patients.

Background: Comorbidity remains a matter of international interest, given growing prevalence of chronic conditions. Objective: To evaluate the impact that adding a telephone coaching intervention by a family physician to usual care has on reducing resource consumption and improving health status, caregiver burden and quality of life among complex chronic patients (CCP) compared with usual care. Methods: A randomized controlled trial was conducted on a random sample of CCP from three primary care teams in Barcelona. Patients were randomly allocated into intervention or control groups. Evaluations were conducted at baseline and after six-month follow-up. Intervention patients were phoned twice a month by a family physician. Both groups received usual care. Primary endpoint was change in total number of urgent visits per patient. Secondary endpoints were changes in health and mental status, quality of life and caregiver burden. Results: Hundred and sixty-one CCP were included. During follow-up, 9 patients died and 2 were lost. At baseline, patients' characteristics and resource consumption were similar for both groups. After six months, urgent visits per patient decreased in intervention (1.27 baseline versus 0.89 follow-up, P = 0.091) and control (1.06 baseline versus 0.86 follow-up, P = 0.422) groups, mean difference 0.18 [confidence interval (CI) 95% -0.48 to 0.84]. Intervention patients improved in the physical component of the SF-12 questionnaire, while worsening in control patients, mean difference 4.71 (CI 95% -9.03 to -0.41). Differences were not found in the rest of the endpoints. Conclusion: The intervention did not reduce urgent visits among CCP neither improved patient's health.

Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sjögren syndrome: analysis of 515 patients.

OBJECTIVE: To evaluate the fulfilment of classification criteria for cryoglobulinaemic vasculitis (CV) at diagnosis in a large cohort of patients with primary SS and their correlation with poor outcomes. METHODS: We included 515 consecutive patients tested for serum cryoglobulins who fulfilled the 2002 classification criteria for primary SS. CV classification criteria and serum cryoglobulins at diagnosis were assessed as predictors of death and lymphoma using Cox proportional-hazards regression analysis adjusted for age and gender. RESULTS: Positive serum cryoglobulins were detected in 65 (12%) patients, of whom 21 (32%) fulfilled CV classification criteria. Compared with patients positive for cryoglobulins who did not fulfil CV criteria, patients with CV had a higher frequency of type II cryoglobulinaemia (86% vs 43%, P = 0.04), a higher mean cryocrit level (6.58% vs 1.25%, P < 0.001) and a higher cumulated mean EULAR-SS disease activity index score (35.3 vs 16.2, P < 0.001). After a mean follow-up of 110 months, 45 (9%) patients developed B-cell lymphoma and 33 (6%) died. Compared with patients without cryoglobulins, patients with cryoglobulins who fulfilled [hazard ratio (HR) = 7.47, 95% CI: 3.38, 16.53] and did not fulfil (HR = 2.56, 95% CI: 1.03, 6.35) CV criteria both showed a higher risk of B-cell lymphoma in the univariate analysis, but not in the multivariate models. Compared with patients without cryoglobulins, patients with CV had a higher risk of death in both the univariate (HR = 11.68, 95% CI: 4.44, 30.74) and multivariate (HR = 4.36, 95% CI: 1.32, 14.47) models. CONCLUSION: Patients with primary SS who fulfilled criteria for cryoglobulinaemic vasculitis at diagnosis are at higher risk of death.

Epidemiologic patterns of disease expression in sarcoidosis: age, gender and ethnicity-related differences.

OBJECTIVES: To evaluate the influence of age, gender and ethnicity in the clinical presentation of sarcoidosis in a cohort of Spanish patients. METHODS: We analysed 175 consecutive patients diagnosed with sarcoidosis between 1990 and 2014 in the Hospital Clinic of Barcelona, Spain. Sarcoidosis was diagnosed according to the 1999 WASOG criteria. Organ involvement was defined using the 2014 WASOG organ assessment instrument. RESULTS: There were 110 women and 65 men, with a mean age at diagnosis of 47.31 ± 15.46 years (range, 16-92); 23% of patients were born outside Spain. Women had a higher mean age (p=0.027), a higher frequency of cutaneous (OR=2.28) and musculoskeletal (OR=2.73) symptoms at diagnosis, and a lower frequency of cumulated WASOG involvements including renal involvement (OR=0.17), hypercalcaemia (OR=0.20) and raised ACE levels (OR=0.30). Patients aged ≥65 years had a lower frequency of cutaneous (OR=0.23) and musculoskeletal (OR=0.13) symptomatology at diagnosis and a higher frequency of cumulated WASOG involvements including renal involvement (OR=18.70) and calcium/vitamin D abnormalities (OR=5.31). According to ethnicity, non-Spanish-born patients had a lower mean age (40 vs. 49 years, p=0.001), a higher predominance of females (68% vs. 54%, p=0.036) and a higher frequency of radiographic stages I/II at diagnosis (97% vs. 78%, p=0.041) in comparison with Spanish-born patients. CONCLUSIONS: Using the new 2014 WASOG organ assessment instrument, we found that epidemiological features (age at diagnosis, gender and ethnicity) play a significant role in the presentation of sarcoidosis. Variations in these epidemiological features may aid early diagnostic suspicion, the search for histopathological confirmation and the prompt introduction of the appropriate therapy.

Circulating SFRP5 levels are elevated in drug-naïve recently diagnosed type 2 diabetic patients as compared with prediabetic subjects and controls.

BACKGROUND: Secreted frizzled-related protein 5 (SFRP5) has been linked to obesity. Results are conflicting regarding its association with type 2 diabetes (T2D) in humans. We aimed to investigate circulating SFRP5 in prediabetes and T2D and its potential association with parameters of insulin resistance and beta-cell function. METHODS: We studied 70 drug-naïve T2D patients, 70 prediabetic subjects and 70 controls. All subjects were body mass index matched to the T2D patients and overweight or obese. SFRP5, hormones and cytokines levels were measured by ELISA. RESULTS: Serum SFRP5 levels were elevated in T2D patients as compared with prediabetic subjects (median 15.6, interquartile range [9-24.5] ng/mL vs 9.8 [5-14.2] ng/mL, p < 0.001, respectively) and controls (15.6 [9-24.5] ng/mL vs 10.4 [6.7-16.6] ng/mL, P < 0.001, respectively). No differences were found in serum SFRP5 levels between prediabetic subjects and controls (9.8 [5-14.2] ng/mL vs 10.4 [6.7-16.6] ng/mL, p = 0.472, respectively). After adjusting for potential confounders (age, gender, body mass index, triglycerides, high-density lipoprotein cholesterol and blood pressure), T2D was still associated with higher values of SFRP5 as compared with prediabetes in multinomial logistic regression analysis (fully adjusted odds ratio 3.50, 95% confidence interval 1.40-8.79, p = 0.008). The association was more subtle when comparing T2D with normal glucose tolerance state (fully adjusted odds ratio 2.18, 95% confidence interval 0.91-5.21, p = 0.078). CONCLUSIONS: Circulating SFRP5 levels were independently associated with T2D as compared with prediabetes and normal glucose tolerance state.