EARLY: a pilot study on early diagnosis of atrial fibrillation in a primary healthcare centre.

AIM: Atrial fibrillation (AF) is associated with high morbidity and mortality. Early diagnosis is likely to improve therapy and prognosis. The study objective was to evaluate the usefulness of a programme for early diagnosis of AF in patients from an urban primary care centre. METHODS AND RESULTS: Participants were recruited from a randomized sample of patients not diagnosed with AF but having relevant risk factors: age ≥ 65 years, ischaemic and/or valvular heart disease, congestive heart failure, hypertension, and/or diabetes. Patients were randomly assigned to the intervention group (IG) or control group (CG). The intervention included (i) initial visit with clinical history, electrocardiogram, and instruction about pulse palpation and warning signs and (ii) electrocardiogram every 6 months during a 2-year follow-up. The main endpoint of the study was the proportion of new cases diagnosed at 6 months. Secondary endpoints were number of new AF diagnoses and complications associated with the arrhythmia in both groups. A total of 928 patients were included (463 IG and 465 CG). At 6 months, AF was diagnosed in 8 IG patients and 1 CG patient (1.7 vs. 0.2%, respectively, P = 0.018). After 2 years of follow-up, 11 IG patients and 6 CG patients had newly diagnosed AF (2.5 vs. 1.3%, respectively, P = 0.132). Time to first diagnosis of AF was shorter in IG patients [median (inter-quartile range): 7 (192) days vs. 227 (188.5) days in CG, P = 0.029]. CONCLUSION: The simple screening proposed could be useful for the early detection of AF in primary care.

Classification and characterisation of peripheral neuropathies in 102 patients with primary Sjögren's syndrome.

OBJECTIVES: This paper aims to analyse the etiology, characterisation and outcomes of the different types of peripheral neuropathy in patients with primary Sjögren's syndrome (SS) and their association with clinical and immunological disease expression. METHODS: A total of 563 consecutive patients diagnosed with primary SS were evaluated. We retrospectively assessed the results of nerve conduction studies carried out in patients with suspected peripheral nervous system involvement. Peripheral neuropathies were classified into mononeuropathy, mononeuropathy multiplex, polyneuropathy and neuronopathy according to the patterns evidenced by electrodiagnostic studies. RESULTS: Nerve conduction studies were carried out in 158/563 (28%) SS patients. The results were normal in 49 and abnormal in 109 patients, in whom peripheral neuropathy was diagnosed in 102. After excluding patients with neuropathy associated with other diseases and patients with entrapment mononeuropathies, 55/563 (10%) patients were classified as having SS-related peripheral neuropathy, including axonal sensorimotor polyneuropathy (n=24), pure sensory neuronopathy (n=15), mononeuropathy multiplex (n=15) and demyelinating polyradiculoneuropathy (n=1). In spite of therapy, clinical progression measured by the MOHS scale was observed in 12% of patients with axonal polyneuropathy, 13% of those with mononeuropathy multiplex and 47% of those with neuronopathy. Survival was significantly reduced in patients with peripheral neuropathy (especially in those with mononeuropathy multiplex and axonal polyneuropathy) in comparison with the control group (log rank =0.001). CONCLUSIONS: We found a prevalence of SS-related peripheral neuropathy of 10%. Classification of neuropathy according to the clinical presentation and electrodiagnostic tests may be useful in determining the functional outcome, therapeutic response and survival.

Forumclínic: the shaping of virtual communities to assist patients with chronic diseases.

Information and communication technologies (ICTs) provide new opportunities to complement traditional care while enhancing patient autonomy. With the objective to supplement patient care, a group of health professionals at the Hospital Clínic de Barcelona created Forumclínic, an online networking website in Spanish and Catalan. In 2008, seven web- and DVD-based chronic disease portals (Diabetes, Schizophrenia, Cardiac Ischemia, COPD, Depression, Breast Cancer and cardiovascular risk) were created with the following resources: multimedia patient education material; physician-specialist transcribed research (articles) news; an open question forum (for clinician-user and user-to-user interaction); and patient and specialist interview videos on the progress of disease, common diagnosis and treatment procedures; and information on the best or worst prognoses. Using data from Google Analytics, server logs were used to observe online behaviour patterns and user postings. This data combined with a mixed methods approach were used to evaluate the development of a virtual community (VC). A virtual community was developed when the number of forum visits was greater than those in the disease portal (definition). While nearly half of the visitors were from the Americas, the Schizophrenia, Breast Cancer, Depression and COPD forums met the criteria for and developed a virtual community. However, the Diabetes and Cardiac Ischemia forums did not reach VC status. It is also interesting to note that users in their late thirties and early forties were primarily women. The development of four virtual communities in Forumclínic seems to support the self-care needs of virtual patients. Users also reported appreciating the increased interaction with experts online and commonly collaborated with the forum moderator to guide and support other users with similar conditions in managing their health. Thence, we believe that Forumclínic is a good model to complement traditional patient care. A formal evaluation of this adjuvant form of care, from both the users' and moderators' perspective, is currently in its final stages.

Gender differences in GPs' strategies for coping with the stress of the COVID-19 pandemic in Catalonia: A cross-sectional study.

BACKGROUND: The Covid-19 pandemic has increased stress levels in GPs, who have resorted to different coping strategies to deal with this crisis. Gender differences in coping styles may be contributing factors in the development of psychological distress. OBJECTIVES: To identify differences by gender and by stress level in coping strategies of GPs during the Covid-19 pandemic. METHODS: A cross-sectional, web-based survey conducted with GPs in Catalonia (Spain), in June-July 2021. via the institution's email distribution list, all GPs members of the Catalan Society of Family and Community Medicine were invited to complete a survey assessing sociodemographic, health and work-related characteristics, experienced stress (Stress scale of the Depression, Anxiety and Stress Scales-DASS 21) and the frequency of use of a range of coping strategies (Brief-COPE) classified as problem-focused, emotion-focused and avoidant strategies, some of which are adaptive and others maladaptive. We compared the scores of each strategy by gender and stress level using Student's t-test. RESULTS: Of 4739 members, 522 GPs participated in the study (response rate 11%; 79.1% women; mean age = 46.9 years, SD = 10.5). Of these, 41.9% reported moderate-severe stress levels. The most common coping strategies were acceptance, active coping, planning, positive reframing and venting. More frequently than men, women resorted to emotional and instrumental support, venting, distraction and self-blame, whereas men used acceptance and humour more commonly than women. Moderate-severe stress levels were associated with non-adaptive coping, with increased use of avoidance strategies, self-blame, religion and venting, and decreased use of positive reframing and acceptance. CONCLUSION: The most common coping strategies were adaptive and differed by gender. However, highly stressful situations caused maladaptive strategies to emerge.

Monoclonal gammopathy related to Sjögren syndrome: a key marker of disease prognosis and outcomes.

OBJECTIVE: To analyze the monoclonal expression of SS through the detection of serum monoclonal immunoglobulins (mIgs) in a large series of patients with Sjögren syndrome (SS), focusing on the etiology, characterization and evolution of the monoclonal band and the association with SS clinical expression and outcomes. METHODS: Serum immunoelectrophoresis (IE) was performed to 408 consecutive patients who were evaluated by our unit between 1992 and 2011: 221 patients who fulfilled the 2002 American-European criteria for primary SS, 122 primary SS patients who fulfilled exclusively the 1993 European criteria and 65 patients with SS-associated hepatitis C virus infection. IE was performed at diagnosis and every year during the follow-up. RESULTS: Of the 221 patients with primary SS, 48 (22%) had monoclonal gammopathy. In the control groups, the prevalence was 16% in patients with SS who fulfilled the 1993 criteria (p > 0.05) and 52% in SS-HCV patients (p < 0.001). Monoclonal bands were characterized in 47/48 patients with primary SS: IgG (n = 21), IgM (n = 16), IgA (n = 5) and free light chains (n = 5); the light chain was κ in 28 patients and λ in 19 (κ:λ ratio 1.5). Primary SS patients with monoclonal gammopathy had a higher prevalence of parotidomegaly (38% vs 20%, p = 0.021), vasculitis (21% vs 6%, p = 0.003), neurological involvement (42% vs 23%, p = 0.016), higher mean values of circulating gammaglobulins (23.4 vs 20.6%, p = 0.026), ESR (56.6 vs 37.6 mm/h, p = 0.003), a higher prevalence of RF (69% vs 50%, p = 0.022), low C3 levels (24% vs 11%, p = 0.028), low C4 levels (24% vs 7%, p = 0.003), low CH50 activity (28% vs 11%, p = 0.008) and cryoglobulins (23% vs 8%, p = 0.012) compared with those without monoclonal gammopathy. Of the 48 patients with primary SS and monoclonal gammopathy, 8 developed hematologic neoplasia after a mean follow-up of 10 years, a higher prevalence than observed in patients without monoclonal gammopathy (17% vs 5%, p = 0.009). Survival rates according to the presence or absence of monoclonal gammopathy were 83% and 97%, respectively (log rank 0.004). CONCLUSION: Monoclonal gammopathy was detected in 22% of patients with primary SS fulfilling the 2002 criteria, with mIgGκ being the most frequent type of band detected. In HCV-associated SS patients, the prevalence was higher (52%) with IgMκ being the most prevalent band detected. Monoclonal gammopathy was associated with a higher prevalence of parotid enlargement, extraglandular features, hypergammaglobulinemia, cryoglobulinemia and related markers (rheumatoid factor, hypocomplementemia), and with a poor prognosis (development of neoplasia and death).

Anti-Ro52 antibody testing influences the classification and clinical characterisation of primary Sjögren's syndrome.

OBJECTIVES: To evaluate how determination of antibodies against the Ro52 antigen influences the classification and clinical characterisation of patients with suspected primary Sjögren's syndrome (SS). METHODS: The cohort study included 187 patients who fulfilled at least four of the six 1993 SS classification criteria, including positive autoantibodies (antinuclear antibodies [ANA], rheumatoid factor [RF], anti-Ro/SSA and/or anti-La/SS-B antibodies) as mandatory criterium. Anti-Ro/SSA antibodies were tested by qualitative ELISA using a commercial assay. Anti-Ro52 antibodies were detected by a semiquantitative ELISA. RESULTS: Anti-Ro52 antibodies were found in 70/187 (37%) patients. A significant percentage of patients with anti-Ro/SSA antibodies were negative for anti-Ro52 antibodies (22%), while 13 patients (12%) were negative for anti-Ro/SSA antibodies but positive for anti-Ro52 antibodies, meaning that they fulfilled the 2002 SS criteria while avoiding the need for a salivary biopsy. Higher mean titers of anti-Ro52 antibodies were associated with severe scintigraphic involvement, positive salivary gland biopsy, parotid enlargement, anaemia, leukopenia and RF. A statistical correlation was found between anti-Ro52 titers and age, gammaglobulin levels, RF titers and serum IgA and IgG. Patients with positive anti-Ro/SSA and anti-Ro52 antibodies had a higher frequency of positive salivary gland biopsy, parotid enlargement and positive RF, and higher levels of serum IgG and IgA levels in comparison with patients with positive anti-Ro/SSA but negative anti-Ro52 antibodies. CONCLUSIONS: Anti-Ro52 antibodies were closely associated with the main clinical, histopathological and immunological features of primary SS. Anti-Ro52 autoantibody testing may help to identify a specific subset of SS patients with more aggressive disease, in whom a closer follow-up and earlier, more robust therapeutic management may be necessary.

Treatment of primary Sjögren syndrome: a systematic review.

CONTEXT: A variety of topical and systemic drugs are available to treat primary Sjögren syndrome, although no evidence-based therapeutic guidelines are currently available. OBJECTIVE: To summarize evidence on primary Sjögren syndrome drug therapy from randomized controlled trials. DATA SOURCES: We searched MEDLINE and EMBASE for articles on drug therapy for primary Sjögren syndrome published between January 1, 1986, and April 30, 2010. STUDY SELECTION: Controlled trials of topical and systemic drugs including adult patients with primary Sjögren syndrome were selected as the primary information source. RESULTS: The search strategy yielded 37 trials. A placebo-controlled trial found significant improvement in the Schirmer and corneal staining scores, blurred vision, and artificial tear use in patients treated with topical ocular 0.05% cyclosporine. Three placebo-controlled trials found that pilocarpine was associated with improvements in dry mouth (61%-70% vs 24%-31% in the placebo group) and dry eye (42%-53% vs 26%). Two placebo-controlled trials found that cevimeline was associated with improvement in dry mouth (66%-76% vs 35%-37% in the placebo group) and dry eye (39%-72% vs 24%-30%). Small trials (<20 patients) found no significant improvement in sicca outcomes for oral prednisone or hydroxychloroquine and limited benefits for immunosuppressive agents (azathioprine and cyclosporine). A large trial found limited benefits for oral interferon alfa-2a. Two placebo-controlled trials of infliximab and etanercept did not achieve the primary outcome (a composite visual analog scale measuring joint pain, fatigue, and dryness); neither did 2 small trials (<30 patients) testing rituximab, although significant results were observed in some secondary outcomes and improvement compared with baseline. CONCLUSIONS: In primary Sjögren syndrome, evidence from controlled trials suggests benefits for pilocarpine and cevimeline for sicca features and topical cyclosporine for moderate or severe dry eye. Anti-tumor necrosis factor agents have not shown clinical efficacy, and larger controlled trials are needed to establish the efficacy of rituximab.

[Fibromyalgia classification: are there different classes of patients?]. / Clasificación de la fibromialgia: existen distintos tipos de pacientes?

Fibromyalgia (FM) is one of the most frequent diseases causing chronic pain. Due to the heterogeneity of its clinical expression and the lack of standardized instruments to classify the signs and symptoms, the availability of a classification system would allow more homogeneous groups of patients to be identified and would permit individualizing diagnostic and therapeutic management. Correct classification of patients with FM requires individual diagnostic evaluation by a multidisciplinary team comprising the family doctor, a specialist in rheumatology or autoimmune diseases, a trauma doctor and a psychologist/psychiatrist. The diagnostic contribution of each specialist together with analysis of the timeline of appearance of signs and symptoms is essential in order to decide to which FM subgroup the patient belongs.

Effectiveness and cost-effectiveness of Improving clinicians' diagnostic and communication Skills on Antibiotic prescribing Appropriateness in patients with acute Cough in primary care in CATalonia (the ISAAC-CAT study): study protocol for a cluster randomised controlled trial.

BACKGROUND: Despite their marginal benefit, about 60% of acute lower respiratory tract infections (ALRTIs) are currently treated with antibiotics in Catalonia. This study aims to evaluate the effectiveness and efficiency of a continuous disease-focused intervention (C-reactive protein [CRP]) and an illness-focused intervention (enhancement of communication skills to optimise doctor-patient consultations) on antibiotic prescribing in patients with ALRTIs in Catalan primary care centres. METHODS/DESIGN: A cluster randomised, factorial, controlled trial aimed at including 20 primary care centres (N = 2940 patients) with patients older than 18 years of age presenting for a first consultation with an ALRTI will be included in the study. Primary care centres will be identified on the basis of socioeconomic data and antibiotic consumption. Centres will be randomly assigned according to hierarchical clustering to any of four trial arms: usual care, CRP testing, enhanced communication skills backed up with patient leaflets, or combined interventions. A cost-effectiveness and cost-utility analysis will be performed from the societal and national healthcare system perspectives, and the time horizon of the analysis will be 1 year. Two qualitative studies (pre- and post-clinical trial) aimed to identify the expectations and concerns of patients with ALRTIs and the barriers and facilitators of each intervention arm will be run. Family doctors and nurses assigned to the interventions will participate in a 2-h training workshop before the inception of the trial and will receive a monthly intervention-tailored training module during the year of the trial period. Primary outcomes will be antibiotic use within the first 6 weeks, duration of moderate to severe cough, and the quality-adjusted life-years. Secondary outcomes will be duration of illness and severity of cough measured using a symptom diary, healthcare re-consultations, hospital admissions, and complications. Healthcare costs will be considered and expressed in 2021 euros (year foreseen to finalise the study) of the current year of the analysis. Univariate and multivariate sensitivity analyses will be carried out. DISCUSSION: The ISAAC-CAT project will contribute to evaluate the effectiveness and efficiency of different strategies for more appropriate antibiotic prescribing that are currently out of the scope of the actual clinical guidelines. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03931577.

Burnout en los médicos de familia de Cataluña durante la quinta ola de la pandemia COVID-19 / Burnout among Catalan family doctors during the fifth wave of the COVID-19 pandemic

Objetivo: la pandemia de la COVID-19 ha tenido un impacto psicológico en los profesionales sanitarios, a menudo manifestándose como burnout. Nuestro objetivo fue estimar la prevalencia del burnout en médicas y médicos de familia de Cataluña durante la pandemia e identificar factores sociodemográficos, laborales y de salud mental asociados.Métodos: estudio descriptivo transversal basado en una encuesta online realizada entre junio y julio de 2021. Se invitó a participar a los 4.700 socios y socias de la Societat Catalana de Medicina Familiar i Comunitària (CAMFiC) y se obtuvo un 11% de respuesta (n = 522).Medidas principales: Maslach Burnout Inventory, con tres dimensiones: agotamiento emocional, despersonalización y realización personal. Análisis bivariante y regresión logística múltiple (variable dependiente: nivel elevado de afectación para cada dimensión de burnout).Resultados: el 67,5% de encuestados presentó niveles altos de agotamiento emocional, el 42,7% de despersonalización y el 29,9% de niveles bajos de realización personal. La prevalencia de agotamiento emocional elevado fue mayor entre las mujeres y los expuestos a pacientes con COVID-19. La edad y años de antigüedad laboral se asoció de forma inversa a agotamiento emocional y despersonalización alta. En el análisis multivariante, la depresión se asoció a despersonalización alta y realización personal baja, la ansiedad a agotamiento emocional alto, y el estrés a las tres dimensiones.Conclusiones: después de 1 año de pandemia, existen niveles elevados de burnout en los médicos y médicas de familia, particularmente en la dimensión de agotamiento emocional. Son necesarias medidas organizativas para proteger la salud mental de las/los profesionales.(AU)

Aim: the COVID-19 pandemic has had a psychological impact on health professionals, often manifesting as burnout. Our purpose was to estimate the prevalence of burnout in family doctors in Catalonia during the pandemic and to identify associated sociodemographic, occupational and mental health factors.Methods: cross-sectional descriptive study based on an online survey conducted June-July 2021. The 4700 members of the Catalan Society of Family and Community Medicine were invited to take part. An 11% response was obtained (n=522).Primary endpoints: Maslach Burnout Inventory, with three dimensions: emotional exhaustion, depersonalization and personal accomplishment. Bivariate analysis and multiple logistic regression (dependent variable: high level of affectation for each area dimension of burnout).Results: a total of 67.5%, 42.7% and 29.9% of respondents presented high levels of emotional exhaustion, depersonalization and low sense of personal accomplishment, respectively. The prevalence of high emotional exhaustion was higher among women and those exposed to COVID-19 patients. Age and seniority were inversely associated with emotional exhaustion and high depersonalization. Multivariate analysis revealed that depression was associated with high depersonalization and low personal accomplishment, anxiety with high emotional exhaustion, and stress with all three areas.Conclusions: One year after onset of the pandemic, we detected high levels of burnout in family doctors, particularly in terms of emotional exhaustion. Organizational measures are necessary to protect the mental health of professionals.(AU)

[Factors predicting a short length of stay for acute exacerbations of chronic obstructive pulmonary disease]. / Factores predictivos de un ingreso corto en pacientes con enfermedad pulmonar obstructiva crónica agudizada.

BACKGROUND AND OBJECTIVE: We intended to identify the factors predicting a short length of stay in patients with chronic obstructive pulmonary disease (COPD) and acute exacerbations in order to select those who are tributary to be admitted in a short stay unit. PATIENTS AND METHOD: Several clinical and biochemical characteristics were compared in a group of patients with COPD admitted in a short stay unit during an acute exacerbation, classified as lasting 4 days or > 4 days of hospital stay. RESULTS: A comorbidity index of 2 [1-2] vs 3 [2-3.5], a number of breaths per minute of 29.0 (6.5) vs 33.6 (6.8) and a pCO2 value on admission of 39.1 (5.3) vs 50.7 (12.0) mmHg were factors independently associated with a shorter stay (p < 0.001 in all). CONCLUSIONS: Some clinical characteristics of COPD patients are predictive of a short length of stay during acute exacerbations.

[Appropriateness of hospitalization in a short stay unit of a teaching hospital. A controlled study]. / Adecuación de la hospitalización en una unidad de estancia corta de un hospital universitario. Un estudio controlado.

BACKGROUND AND OBJECTIVE: Our aim was to evaluate the rate and the reasons for inappropriate hospital use in a medical Short Stay Unit (SSU) in comparison with conventional hospitalization. PATIENTS AND METHOD: The Appropriateness Evaluation Protocol was applied to 352 patients admitted in the SSU and to 203 patients admitted in a department of internal medicine (control group). RESULTS: The rates of inappropriate hospital admissions (0.6% vs 3%; p = 0.023) and stays (6.9% vs 18.7%; p < 0.001) were significantly lower in the SSU with respect to the control group. Main causes of inappropriateness were also lower: conservative physician's attitude (9.4% vs 41.4%; p = 0.001), delay in diagnostic procedures (3.4% vs 11.3%; p < 0.001), and social and familiar factors (2.8% vs 9.4%; p = 0.001). CONCLUSIONS: A SSU is useful for the appropriateness of hospital use, due to its capacity to minimize the number and causes of inappropriate stays.

Diagnosis of Liver Involvement in Primary Sjögren Syndrome.

Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sjögren syndrome (SS). In the 1990s, a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease, liver function and a complete panel of autoantibodies. Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS. The most recent studies have shown that, after eliminating hepatotoxic drugs and fatty liver disease, the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases. The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important, since the two processes require different therapeutic approaches and have different prognoses. With respect to viral infections, chronic HCV infection is the main cause of liver involvement in SS patients from the Mediterranean area, while chronic HBV infection may be the main cause of liver involvement in SS patients from Asian countries. After eliminating viral hepatitis, primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS. PBC-related SS patients may have a broad spectrum of abnormalities of the liver, including having no clinical or analytical data suggestive of liver disease. Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I), and nearly 10% of these patients have an AIH-PBC overlap. Finally, IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis, especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present.

[Adverse effects of antiarrhythmic drugs in patients with atrial fibrillation in primary care]. / Estudio sobre los efectos adversos de los fármacos antiarrítmicos en pacientes con fibrilación auricular atendidos en un Centro de Atención Primaria.

BACKGROUND AND OBJECTIVE: To determine the adverse effects (AE) of antiarrhythmic drugs used to treat atrial fibrillation (AF) in routine clinical practice. PATIENTS AND METHODS: A retrospective, observational, population-based study of all patients diagnosed of AF requiring long-term treatment with antiarrhythmic drugs. We performed also a descriptive analysis of the type of EA, and their management (diagnostic techniques, laboratory tests, drug treatment, number of visits to health services, emergency visits, hospitalizations and surgeries). RESULTS: We included 400 patients. Overall, 688 treatments were started, 34 of which (4.9%) were implied in 32 EA in 30 patients. The most frequent EA were hypothyroidism (28.1%), hyperthyroidism (25%) and bradycardia (9.38%). In absolute numbers, class III drugs (Vaughan-Williams classification) were associated with a higher number of AE (p=0.001). The number of AE/100 patients-year was: class I 0.42 (0.01-1.15%), class II 2.55 (0.68-5.13%), class III 4.70 (2.98-6.64%), class IV 1.18 (0.14-2.80%), digoxin 0.41 (0.08-1.19). Individually, the drugs with a higher rate were sotalol, bisoprolol and amiodarone (5.70, 5.37 and 4.59 respectively). 93.75% of EA required visits to health services, 59.38% laboratory tests and 56.25% diagnostic techniques. The most frequent visits were scheduled for primary care (75%), followed by scheduled visits to outpatient cardiology (21.9%). 71.9% of EA were resolved without any sequel and one patient died. CONCLUSIONS: Antiarrhythmic drugs for the management of AF are associated with the presence of AE, mainly class III drugs. In most cases they are not serious, but imply important health resources.

Clinical and prognostic significance of parotid scintigraphy in 405 patients with primary Sjogren's syndrome.

OBJECTIVE: To evaluate the association between the degree of involvement shown by parotid scintigraphy at diagnosis and the disease expression, outcomes, and prognosis of primary Sjögren's syndrome (SS). METHODS: All patients consecutively diagnosed with primary SS in our department between 1984 and 2008 were evaluated. The scintigraphic stages were classified into class 4 (severe involvement), class 2-3 (mild to moderate involvement), and class 1 (normal results). RESULTS: A total of 405 patients with primary SS underwent parotid scintigraphy at diagnosis (47 had class 1 involvement, 314 had class 2-3, and 44 had class 4). Patients with class 4 had a higher frequency of parotid enlargement (p < 0.001), systemic involvement (p = 0.007), high titers of antinuclear antibody (p = 0.016), positive rheumatoid factor (p = 0.002), anti-Ro/SSA (p = 0.001), anti-La/SSB (p = 0.001), low C4 levels (p = 0.001), and low CH50 (p = 0.001) in comparison with the other 2 groups. A higher rate of lymphoma development was observed in patients with class 4 involvement. Adjusted multivariate Cox regression analysis showed a hazard ratio (HR) of 10.51 (p = 0.002) and Kaplan-Meier analysis a log-rank of 0.0005. Mortality was 5-fold higher in patients with class 4 involvement. Adjusted multivariate Cox regression analysis showed an HR of 5.33 (p = 0.001) and Kaplan-Meier analysis a log-rank of 0.033. CONCLUSION: Patients with SS presenting with severe scintigraphic involvement at diagnosis had a more pronounced autoimmune expression, a higher risk of developing systemic features and lymphoma, and a lower survival rate. Study of the degree of salivary gland dysfunction at diagnosis by parotid scintigraphy offers valuable clinical information on the prognosis and outcome of primary SS.

Outcomes in biopsy-proven lupus nephritis: evaluation of 190 white patients from a single center.

We describe the natural history of lupus nephritis (LN) in a historical cohort of 190 white patients with the diagnosis of biopsy-proven LN followed in a single reference center.We evaluated 670 patients with systemic lupus erythematosus (SLE) consecutively followed in our department from 1970 until 2006. All patients fulfilled the 1997 revised criteria for the classification of SLE. White patients (Spanish-born) with biopsy-proven LN were selected as the study population.The cohort included 190 patients (170 female patients and 20 male) with a mean age at LN diagnosis of 31 years. Renal biopsy revealed type I LN in 8 (4%) patients, type II in 33 (17%), type III in 46 (24%), type IV in 72 (38%), type V in 28 (15%), and type VI in 3 (2%) patients. Induction remission was achieved in 85% of patients with types I and II, 78% with type III, 70% with type IV, and 32% of patients with type V. After a mean follow-up of 2391 patient-years, 62 (33%) patients developed chronic renal failure and 18 (9%) evolved to end-stage renal disease. Adjusted multivariate Cox regression analysis identified male sex (hazard ratio [HR], 4.33) and elevated creatinine at LN diagnosis (HR, 5.18) as independent variables for renal failure. Survival was 92% at 10 years of follow-up, 80% after 20 years, and 72% after 30 years.Our results suggest that biopsy-proven LN in white patients has an excellent prognosis. Ethnicity should be considered a key factor when evaluating the prognosis and therapeutic response to different agents in patients with LN.

Sjögren syndrome or sjögren disease? The histological and immunological bias caused by the 2002 criteria.

The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren "disease." In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren "syndrome" seems more adequate.

[Classification of fibromyalgia. A systematic review of the literature]. / Clasificación de la fibromialgia. Revisión sistemática de la literatura.

OBJECTIVE: To review the scientific literature concerning the classification of fibromyalgia (FM), including previous studies focusing on the gnosologic evaluation of FM, with the aim of proposing a classificatory hypothesis based on the current scientific evidence. METHODS: Systematic review using a baseline MEDLINE search. Search terms included «fibromyalgia¼ and «classification¼. Additional articles were identified through a comprehensive manual search of the references of retrieved articles. RESULTS: This systematic review has identified, on the one hand, several classificatory proposals based on psychopathological aspects, and, on the other hand, the key role of associated diseases. Based on the scientific evidence currently available, the following FM subsets were defined: patients with no associated processes (type I FM), patients with associated rheumatic/autoimmune chronic diseases (type II FM), patients with severe psychiatric disorders (type III FM) and patients with simulated FM (type IV FM). CONCLUSIONS: Few studies have specifically analysed the classification of FM into subgroups with a more homogeneous clinical expression. Correct classification of patients with FM requires the integration of two key concepts (psychopathological evaluation and coexistence of comorbid processes), with an individual diagnostic evaluation by a multidisciplinary team.

Prevalence and clinical relevance of autoimmune neutropenia in patients with primary Sjögren's syndrome.

OBJECTIVE: To analyze the prevalence of neutropenia in a large cohort of patients with primary Sjögren's syndrome (SS) and its association with clinical and immunological disease expression and adverse outcomes. METHODS: The study cohort included 300 patients diagnosed with primary SS in our department between 1984 and 2002. The outcomes measured after the first laboratory evidence of neutropenia (<2.5 x 10(9)/L) were first hospital admission caused by infection, development of systemic manifestations, neoplasia, and death. RESULTS: Ninety-nine (33%) patients had neutropenia during the follow-up, which was related to neoplasia or drugs in 9 (3%) patients and was considered idiopathic in the remaining 90 (30%). Patients with neutropenia had a lower mean age at diagnosis of SS (51.9 versus 59.4 years, P < 0.001) and a higher prevalence of anti-Ro/La antibodies (53% versus 22%, P < 0.001), rheumatoid factor (49% versus 32%, P = 0.009), and low C4 levels (17% versus 8%, P = 0.044) than those without neutropenia. Patients with neutropenia had a higher incidence of hospital admission caused by infection (24% versus 9%, P = 0.002), especially those with neutropenia <1 x 10(9)/L (50% versus 9%, P = 0.002), and a higher rate of admission (log rank = 0.0023) in comparison with those without neutropenia. Agranulocytosis was found in 7 (2%) patients, predominantly related to neoplasia (5 cases). One (1%) of the 90 patients with SS-related neutropenia developed large granular lymphocyte T-cell leukemia. CONCLUSION: Neutropenia should be considered a relevant hematologic finding of primary SS, due both to its elevated prevalence and to its clinical significance (close association with anti-Ro/La antibodies, coexistence with other cytopenias, and development of severe infections).