Validation of the Spanish version of the fibromyalgia rapid screening tool to detect fibromyalgia in primary care health centres.

OBJECTIVES: To investigate the reliability and validity of the Spanish version of the Fibromyalgia Rapid Screening Tool (FiRST), a brief questionnaire for the detection of fibromyalgia (FM) in patients with diffuse chronic pain seen at primary care health centres. METHODS: The original FiRST French questionnaire was adapted to a Spanish version following the guidelines of the Rheumatology Spanish Society Study Group of FM, and the help provided by professors of French and Spanish Language. In a prospective and multicentre study, patients with chronic pain were initially divided into two groups: a group that included patients that had been diagnosed with FM according to the 1990 ACR criteria and the 2010 ACR preliminary criteria (n=404), and a non-FM (control) group composed of rheumatoid arthritis (RA) (n=147) and osteoarthritis (OA) (n=219) patients. Patients from the FM group were evaluated by assessing tender point assessment, Widespread Pain Index (WPI), Symptom Severity Scale (SSS), FiRST questionnaire and Fibromyalgia Impact Questionnaire (FIQ). The non-FM group was evaluated by means of FiRST, WPI and SSS. Sensitivity, specificity and predictive value as well as the correlation between the global score and other parameters were assessed. RESULTS: 356 of 404 FM (88.1%) patients who met the 1990 ACR criteria and the ACR 2010 preliminary criteria had a positive FiRST. In the control group (AR plus OA), only 16 (4.4%) subjects had a positive FiRST. The sensitivity value was 92% (95% confidence interval CI: 88.9-95.1), specificity 87.4% (95% CI: 80.8-94.0), positive predictive value 95.7% (95% CI: 93.3-98.1), and negative predictive value 78.2% (95% CI: 70.6-85.9). A significant correlation between the total FiRST score (patients with score 5 or 6) and WPI (p<0.0001), SSS (p<0.0001), time to disease progression (p<0.0001) and FIQ (p<0.0001) was found. CONCLUSIONS: FiRST questionnaire is a useful tool for the detection of FM in primary care health centres.

The Spanish version of the 2010 American College of Rheumatology Preliminary Diagnostic Criteria for fibromyalgia: reliability and validity assessment.

OBJECTIVES: To investigate the reliability and validity of the Spanish version of the 2010 American College of Rheumatology (ACR) Preliminary Diagnostic Criteria for Fibromyalgia (FM) in patients with chronic pain. METHODS: The 2010 ACR Preliminary Diagnostic Criteria for FM were adapted to a Spanish version following the guidelines of the Rheumatology Spanish Society Study Group of FM. Based on the 1990 ACR classi cation criteria for FM, patients with chronic pain were initially divided into two groups: a FM group and another group of non-FM individuals. Patients from the FM group were evaluated by tender points (TP) examination, Fibromyalgia Impact Questionnaire (FIQ), Widespread Pain Index (WPI), and Symptom Severity Scale (SSS). The non-FM (control) group included patients with rheumatoid arthritis (RA) and osteoarthritis (OA). They were evaluated by WPI and SSS. RESULTS: We included 1,169 patients divided into two groups: FM group (n=803; 777 women and 26 men) and non-FM group (n= 366; 147 patients with RA, and 219 with OA). The median value of TP and FIQ in the FM group was 16 and 74 respectively. The preliminary 2010 ACR criteria were met by 665 (82.8%) FM patients and by 112 (30.6%) patients from the non-FM group (p<0.0001). Statistically signi cant differences in the number of TP (p<0.03), FIQ (p<0.0001), WPI (p<0.0001) and SSS (p<0.0001) were observed when FM patients fulfilling the 2010 ACR criteria were compared with the remaining FM patients who did not fulfill these criteria. Sensitivity of the Spanish version of the 2010 ACR criteria was 85.6% (95%CI: 83.1-88.1), speci city 73.2% (95%CI: 68.4-78), positive predictive value 87.7% (95%CI: 85.3-90.1) and negative predictive value 69.4% (95%CI: 64.5-74.2). CONCLUSIONS: Our results indicate that the 2010 ACR Preliminary Diagnostic Criteria for FM may be useful to establish a diagnosis of FM in Spanish individuals with chronic pain.

Revisión sistemática del uso de adalimumab en enfermedades autoinmunes. Eficacia y seguridad en 54 pacientes / Systematic review on the use of adalimumab in autoinmune. Efficacy and safety in 54 patients

Objetivo Analizar la experiencia publicada sobre el uso de adalimumab en el tratamiento de enfermedades autoinmunes en adultos. Métodos Se realizó una revisión sistemática de los artículos incluidos en la base de datos Medline desde el 1 de enero de 1990 al 31 de diciembre de 2008, combinando el término «adalimumab» con las diferentes enfermedades autoinmunes sistémicas. Se identificaron un total de 241 artículos, de los que 154 se revisaron a texto completo y 18 fueron finalmente seleccionados como relevantes. Resultados En los 18 artículos seleccionados se incluían 54 pacientes adultos con enfermedades autoinmunes tratados con adalimumab: 16 pacientes con enfermedad de Behçet; 13 con uveítis idiopática; 5 con sarcoidosis; 5 con uveítis asociadas a otras enfermedades (psoriasis en 2, espondilitis anquilosante en 1, artritis idiopática juvenil en 1, enfermedad de Crohn en 1); 4 con enfermedad de Vogt-Koyanagi-Harada; 4 con uveítis de Birdshot; 3 con vasculitis (arteritis de la temporal, enfermedad de Takayasu y una vasculitis cutánea asociada a artritis reumatoide), 2 con enfermedad de Still del adulto; uno con policondritis recidivante y un paciente con esclerosis sistémica. Las manifestaciones clínicas que motivaron la indicación fueron la uveítis (39 casos), afectación mucocutánea (9), vasculitis (3), artritis (6) y afectación pulmonar (3). En todos los casos se trataban de enfermedades refractarias a tratamiento con glucocorticoides (42 casos, 78%), inmunosupresores (42, 78%) y otros biológicos (29, 54%). Cincuenta (93%) pacientes respondieron a adalimumab. La respuesta fue similar tanto en aquellos que habían recibido otro biológico como en los que adalimumab era el primer biológico administrado. En 5 (9%) pacientes se describieron efectos adversos (3 reacciones cutáneas locales, un paciente con angioedema y una exacerbación de una fibrosis pulmonar). Tras un tiempo medio de seguimiento de 11,9 meses, 12 (22%) pacientes presentaron recidiva y uno (2%) falleció por exacerbación de su enfermedad de base. Conclusiones La evidencia del uso de adalimumab en enfermedades autoinmunes proviene de casos aislados y ensayos no controlados, que incluyen en todos los casos a pacientes graves y refractarios a tratamiento convencional. En este contexto clínico, el uso de adalimumab aparece como una opción eficaz y segura, especialmente en pacientes con uveítis y enfermedad de Behçet (AU)

Objective To analyze published evidence about adalimumab use in autoimmune diseases. Methods Systematic review of MEDLINE database of citations included from January 1990 to December 2008 employing the terms “adalimumab” and the different systemic autoimmune diseases. Results Our search identified 241 potentially relevant citations. 154 were retrieved for detailed evaluation. Finally, 18 were selected as relevant, including 54 patients. The reported diseases were as follow: Behçet disease in 16 patients, idiopathic uveitis in 13, sarcoidosis in 5, uveitis associated with rheumatologic diseases in 5 (psoriasis in 2, ankylosing spondylitis in 1, juvenile idiopathic arthritis in 1, Crohn disease in 1), Vogt-Koyanagi-Harada disease in 4, Birdshot uveitis in 4, vasculitis in 3 (1 temporal arteritis, 1 Takayasu′s disease, 1 skin vasculitis associated with rheumatoid arthritis), adult onset Still disease in 2, relapsing polychondritis in 1 and systemic sclerosis in 1. The clinical spectrum included uveitis (39 cases), skin and/or mucosae (9), vasculitis (3), arthritis (6), lung (3). These patients were refractory to standard therapy, including corticosteroids (42 cases, 78%), immunosuppressants (42, 78%) and biologics (29, 54%). Fifty (93%) patients responded to adalimumab. The clinical response was similar in those patients who had been treated with other biologic and in those who had not received biologic therapy before adalimumab. The patients were followed for 11.9 months. Twelve (22%) patients relapsed. Five (9%) patients suffer some side effect (3 local skin reaction, 1 angioedema, 1 lung fibrosis). One patient (2%) died due to progression of her disease. Conclusions Available data about the use of adalimumab in autoinmune diseases come from case reports and uncontrolled studies, that include patients with severe disease and refractory to standard therapy. In this setting, it seems to be an effective and safe treatment option, especially in patients with uveitis and Behçet's disease. This initial data must be confirmed by controlled assays before extending adalimumab use (AU)

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.

[Systematic review on the use of adalimumab in autoinmune. Efficacy and safety in 54 patients]. / Revisión sistemática del uso de adalimumab en enfermedades autoinmunes. Eficacia y seguridad en 54 pacientes.

OBJECTIVE: To analyze published evidence about adalimumab use in autoimmune diseases. METHODS: Systematic review of MEDLINE database of citations included from January 1990 to December 2008 employing the terms "adalimumab" and the different systemic autoimmune diseases. RESULTS: Our search identified 241 potentially relevant citations. 154 were retrieved for detailed evaluation. Finally, 18 were selected as relevant, including 54 patients. The reported diseases were as follow: Behçet disease in 16 patients, idiopathic uveitis in 13, sarcoidosis in 5, uveitis associated with rheumatologic diseases in 5 (psoriasis in 2, ankylosing spondylitis in 1, juvenile idiopathic arthritis in 1, Crohn disease in 1), Vogt-Koyanagi-Harada disease in 4, Birdshot uveitis in 4, vasculitis in 3 (1 temporal arteritis, 1 Takayasu's disease, 1 skin vasculitis associated with rheumatoid arthritis), adult onset Still disease in 2, relapsing polychondritis in 1 and systemic sclerosis in 1. The clinical spectrum included uveitis (39 cases), skin and/or mucosae (9), vasculitis (3), arthritis (6), lung (3). These patients were refractory to standard therapy, including corticosteroids (42 cases, 78%), immunosuppressants (42, 78%) and biologics (29, 54%). Fifty (93%) patients responded to adalimumab. The clinical response was similar in those patients who had been treated with other biologic and in those who had not received biologic therapy before adalimumab. The patients were followed for 11.9 months. Twelve (22%) patients relapsed. Five (9%) patients suffer some side effect (3 local skin reaction, 1 angioedema, 1 lung fibrosis). One patient (2%) died due to progression of her disease. CONCLUSIONS: Available data about the use of adalimumab in autoinmune diseases come from case reports and uncontrolled studies, that include patients with severe disease and refractory to standard therapy. In this setting, it seems to be an effective and safe treatment option, especially in patients with uveitis and Behçet's disease. This initial data must be confirmed by controlled assays before extending adalimumab use.

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

Objetivo: Recopilar la información existente sobre la clasificación de la fibromialgia (FM), analizar los antecedentes relevantes en relación con una subdivisión nosológica y proponer una hipótesis clasificatoria fundamentada en la evidencia científica existente en la actualidad. Metodología: Revisión sistemática de la literatura mediante búsqueda en la página web PubMed. Los términos de búsqueda en MEDLINE fueron «fibromyalgia» y «classification». Se realizó una búsqueda manual adicional entre las referencias de los artículos seleccionados. Resultados: La revisión sistemática de la literatura ha permitido identificar, por una parte, la existencia de varias propuestas clasificatorias esencialmente basadas en criterios psicopatológicos y, por otra parte, la importancia de evaluar las enfermedades relacionadas con la FM. Según la evidencia analizada, se han identificado los siguientes subgrupos clasificatorios en el paciente que cumple los criterios vigentes de 1990: pacientes sin ninguna enfermedad concomitante (FM tipo I), pacientes con enfermedades crónicas reumáticas y autoinmunitarias (FM tipo II), pacientes con grave alteración en la esfera psicopatológica (FM tipo III) y pacientes simuladores (FM tipo IV). Conclusiones: Son pocos los estudios que analizan de forma específica cómo identificar subgrupos de pacientes con FM con una expresión clínica más homogénea. El análisis ha permitido identificar que los principales aspectos a evaluar en la clasificación del paciente con FM son el perfil psicopatológico y la coexistencia de otros procesos. Una adecuada clasificación del paciente con FM sólo puede realizarse mediante una evaluación diagnóstica individualizada por parte de un equipo mutidisciplinario (AU)

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 «fibromyalgia» and «classification». 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 (AU)

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).

[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.

[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.

Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients.

We conducted the current study to characterize the clinical presentation of primary Sjögren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases. The cohort included 937 women and 73 men (ratio, 13:1), with a mean age of 53 years at diagnosis and 59 years at inclusion in the registry. Multivariate analysis showed that male patients had a lower frequency of thyroiditis, Raynaud phenomenon, and antinuclear antibodies. Young-onset patients had a low degree of sicca involvement (xerostomia and parotid enlargement) and a high frequency of immunologic markers (anti-Ro/SS-A and low C4 levels). Patients with disease duration of more than 10 years had a higher prevalence of xerophthalmia, parotid enlargement, lung involvement, and peripheral neuropathy in comparison with incident cases. The subset of patients with anti-Ro/La antibodies had the highest prevalence of most systemic, hematologic, and immunologic alterations (higher frequency of Raynaud phenomenon, altered parotid scintigraphy, positive salivary gland biopsy, peripheral neuropathy, thrombocytopenia, and rheumatoid factor). Hypocomplementemia was associated with a higher frequency of vasculitis and lymphoma, and cryoglobulins with a higher frequency of parotid enlargement, vasculitis, and leukopenia.Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of primary SS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers. Primary SS should be considered as a systemic autoimmune disease that can express in many guises beyond sicca involvement.

High prevalence of serum metabolic alterations in primary Sjögren's syndrome: influence on clinical and immunological expression.

OBJECTIVE: To analyze the prevalence and clinical significance of associated metabolic alterations [dyslipidemia, diabetes mellitus (DM), and hyperuricemia] in a large series of unselected patients with primary Sjögren's syndrome (SS). METHODS: We analyzed 254 consecutive patients with primary SS who had a complete analytical followup study for at least 5 consecutive years. The control group consisted of 254 age and sex-matched patients without systemic autoimmune diseases consecutively followed during the same period in a primary care center. RESULTS: In comparison with controls, patients with primary SS showed a higher frequency of dyslipidemia (47% vs 33%; p = 0.002), DM (28% vs 18%; p = 0.006), and hyperuricemia (9% vs 4%; p = 0.007). The mean age at SS diagnosis was 10 years greater in patients with DM (p < 0.001) and hyperuricemia (p = 0.009). Hypercholesterolemia was associated with a lower frequency of immunological markers such as anti-Ro/SSA antibodies (p = 0.001), anti-La/SSB antibodies (p = 0.005), low C3 (p = 0.047), and low C4 levels (p = 0.030), while hypertriglyceridemia and DM were associated with a higher prevalence of extraglandular features, especially renal, liver, and vasculitic involvement. A higher prevalence of DM was found in patients treated with corticosteroids (40% vs 19%; p = 0.001). CONCLUSION: Patients with primary SS showed a higher prevalence of associated dyslipidemia, DM, and hyperuricemia in comparison with an age and sex-matched control group. Metabolic alterations were associated with a differentiated pattern of clinical and immunological SS expression, but not with SS-related therapies (except for the higher frequency of DM observed in patients treated with corticosteroids).