Multiple Drug Regimen-Refractory Rosai-Dorfman-Destombes Disease Mimicking Relapsing Polychondritis Successfully Treated with Cobimetinib.

Rosai-Dorfman-Destombes disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare non-Langerhans cell histiocytosis of unknown cause. The disease often manifests as painless bilateral cervical lymphadenopathy associated with systemic symptoms such as fever and weight loss. Extranodal disease is also frequent and can involve any organ, mostly the skin, nasal cavity, bone, and retro-orbital tissue. Swelling of cartilaginous tissues, such as the helix of the ear or laryngeal structures, may mimic the entity known as relapsing polychondritis. Although spontaneous remission is the most expected evolution, some cases require systemic treatment with prednisone, methotrexate or cytotoxic agents, with variable rates of success. In this respect, since somatic variants in the genes involved in the mitogen-activated protein kinase (MAPK) and extracellular signal-regulated kinases (ERK) pathway have been observed to play a pathogenic role in RDD. Therefore, the use of therapies targeting these pathogenic variants appears to be a reasonable strategy. Here we present the case of a 37-year-old woman with RDD and extensive extranodal involvement that showed a rapid and complete response to the MEK inhibitor cobimetinib. LEARNING POINTS: Rosai-Dorfman-Destombes disease (RDD) may mimic the entity known as relapsing polychondritis but should be treated with drug therapy for the underlying disease.Mutations in MAPK/ERK pathway components should be determined in RDD with systemic involvement, although testing to determine every somatic mutation responsible for the disease is not available in all healthcare centres.MEK inhibitors like cobimetinib could be effective in RDD cases with severe and refractory systemic disease, even if molecular analysis has not been possible.

Recomendaciones de la Sociedad Española de Reumatología sobre el uso de terapias biológicas en espondiloartritis axial / Recommendations by the Spanish Society of Rheumatology on the Use of Biological Therapies in Axial Spondyloarthritis

Objetivo: La aparición de nueva información sobre las terapias biológicas en la espondiloartritis axial (EspAax) ha impulsado una nueva revisión de las recomendaciones de la Sociedad Española de Reumatología (SER) basadas en la mejor evidencia posible. Estas nuevas recomendaciones pueden servir de referencia para reumatólogos implicados en el tratamiento de estos pacientes. Métodos: Se creó un panel formado por nueve reumatólogos expertos en EspAax, previamente seleccionados por la SER mediante una convocatoria abierta. Las fases del trabajo fueron: identificación de las áreas clave para la actualización del consenso anterior, análisis y síntesis de la evidencia científica (sistema modificado de Oxford, CEBM, 2009) y formulación de recomendaciones a partir de esta evidencia y de técnicas de consenso. Resultados: Esta revisión de las recomendaciones comporta una actualización en la evaluación de actividad de la enfermedad y objetivos de tratamiento. Incorpora también los nuevos fármacos disponibles, así como sus nuevas indicaciones, y una revisión de los factores predictivos de respuesta terapéutica y progresión del daño radiográfico. Finalmente, estas recomendaciones abordan también las situaciones de fracaso a un primer anti-TNF, así como la posible optimización de la terapia biológica. El documento incluye una tabla de recomendaciones y un algoritmo de tratamiento. Conclusiones: Se presenta la actualización de las recomendaciones SER para el uso de terapias biológicas en pacientes con EspAax

Objective: Recent data published on biological therapy in axial spondyloarthritis (axSpA) since the last publication of the recommendations of the Spanish Society of Rheumatology (SER) has led to the generation of a review of these recommendations based on the best possible evidence. These recommendations should be a reference for rheumatologists and those involved in the treatment of patients with axSpA. Methods: Recommendations were drawn up following a nominal group methodology and based on systematic reviews. The level of evidence and grade of recommendation were classified according to the model proposed by the Centre for Evidence Based Medicine at Oxford. The level of agreement was established through the Delphi technique. Results: In this review, we did an update of the evaluation of disease activity and treatment objectives. We included the new drugs with approved therapeutic indication for axSpA. We reviewed both the predictive factors of the therapeutic response and progression of radiographic damage. Finally, we drafted some recommendations for the treatment of patients refractory to anti-tumor necrosis factor, as well as for the possible optimization of biological therapy. The document also includes a table of recommendations and a treatment algorithm. Conclusions: We present an update of the SER recommendations for the use of biological therapy in patients with axSpA

Criterios de cribado de enfermedad inflamatoria intestinal y espondiloartritis para derivación de pacientes entre Reumatología y Gastroenterología / Screening of inflammatory bowel disease and spondyloarthritis for referring patients between Rheumatology and Gastroenterology

Objetivo. Definir criterios clínicos de cribado de espondiloartritis (SpA) en pacientes con enfermedad inflamatoria intestinal (EII) y viceversa, que sirvan de referencia en la derivación entre Reumatología y Aparato Digestivo. Material y métodos. Revisión sistemática de la literatura y Delphi a dos rondas. Formaron parte del comité científico 2 reumatólogos y 2 digestólogos; del panel de expertos, 7 reumatólogos y 7 digestólogos. El comité científico definió los componentes potenciales de los criterios, teniendo en cuenta aspectos de sensibilidad, especificidad, facilidad de uso y estandarización. A continuación, se realizó el Delphi. Aquellos ítems para los que hubo acuerdo en primera o segunda ronda formaron parte de la versión final de los criterios. Resultados. Cribado positivo de SpA si se cumple al menos uno de los siguientes: dolor lumbar crónico con inicio antes de los 45 años; dolor lumbar inflamatorio o dolor alternante en nalgas; HLA-B27 positivo; sacroilitis en pruebas de imagen; artritis; entesitis del talón; dactilitis. Cribado positivo de EII si uno de los criterios mayores o al menos dos de los menores. Mayores: rectorragia; diarrea crónica de características orgánicas; enfermedad perianal. Menores: dolor abdominal crónico; anemia ferropénica o ferropenia; manifestaciones extraintestinales; fiebre o febrícula, sin focalidad aparente y de más de una semana de duración; pérdida de peso no explicable; antecedentes familiares de EII. Conclusiones. Se han definido criterios de cribado de EII en pacientes con SpA y viceversa. Estos han de ser de utilidad en la detección precoz de dichas patologías (AU)

Objective. To define clinical screening criteria for spondyloarthritis (SpA) in patients with inflammatory bowel disease (IBD) and vice versa, which can be used as a reference for referring them to the rheumatology or gastroenterology service. Method. Systematic literature review and a two-round Delphi method. The scientific committee and the expert panel were comprised of 2 rheumatologists and 2 gastroenterologists, and 7 rheumatologists and 7 gastroenterologists, respectively. The scientific committee defined the initial version of the criteria, taking into account sensitivity, specificity, standardization and ease of application. Afterwards, members of the expert panel assessed each item in a two-round Delphi survey. Items that met agreement in the first or second round were included in the final version of the criteria. Results. Positive screening for SpA if at least one of the following is present: onset of chronic low back pain before 45 years of age; inflammatory low back pain or alternating buttock pain; HLA-B27 positivity; sacroiliitis on imaging; arthritis; heel enthesitis; dactylitis. Positive screening for IBD in the presence of one of the major criteria or at least two minor criteria. Major: rectal bleeding; chronic diarrhea with organic characteristics; perianal disease. Minor: chronic abdominal pain; iron deficiency anemia or iron deficiency; extraintestinal manifestations; fever or low grade fever, of unknown origin and duration >1week; unexplained weight loss; family history of IBD. Conclusion. Screening criteria for IBD in patients with SpA, and vice versa, have been developed. These criteria will be useful for early detection of both diseases (AU)

Criterios de cribado de enfermedad inflamatoria intestinal y espondiloartritis para derivación de pacientes entre Reumatología y Gastroenterología / Screening of inflammatory bowel disease and spondyloarthritis for referring patients between rheumatology and gastroenterology

Objetivo: Definir criterios clínicos de cribado de espondiloartritis (SpA) en pacientes con enfermedad inflamatoria intestinal (EII) y viceversa, que sirvan de referencia en la derivación entre Reumatología y Aparato Digestivo. Material y métodos: Revisión sistemática de la literatura y Delphi a dos rondas. Formaron parte del comité científico 2 reumatólogos y 2 digestólogos; del panel de expertos, 7 reumatólogos y 7 digestólogos. El comité científico definió los componentes potenciales de los criterios, teniendo en cuenta aspectos de sensibilidad, especificidad, facilidad de uso y estandarización. A continuación, se realizó el Delphi. Aquellos ítems para los que hubo acuerdo en primera o segunda ronda formaron parte de la versión final de los criterios. Resultados: Cribado positivo de SpA si se cumple al menos uno de los siguientes: dolor lumbar crónico con inicio antes de los 45 años; dolor lumbar inflamatorio o dolor alternante en nalgas; HLA-B27 positivo; sacroilitis en pruebas de imagen; artritis; entesitis del talón; dactilitis. Cribado positivo de EII si uno de los criterios mayores o al menos dos de los menores. Mayores: rectorragia; diarrea crónica de características orgánicas; enfermedad perianal. Menores: dolor abdominal crónico; anemia ferropénica o ferropenia; manifestaciones extraintestinales; fiebre o febrícula, sin focalidad aparente y de más de una semana de duración; pérdida de peso no explicable; antecedentes familiares de EII. Conclusiones: Se han definido criterios de cribado de EII en pacientes con SpA y viceversa. Estos han de ser de utilidad en la detección precoz de dichas patologías (AU)

Objective: To define clinical screening criteria for spondyloarthritis (SpA) in patients with inflammatory bowel disease (IBD) and vice versa, which can be used as a reference for referring them to the rheumatology or gastroenterology service. Method: Systematic literature review and a two-round Delphi method. The scientific committee and the expert panel were comprised of 2 rheumatologists and 2 gastroenterologists, and 7 rheumatologists and 7 gastroenterologists, respectively. The scientific committee defined the initial version of the criteria, taking into account sensitivity, specificity, standardization and ease of application. Afterwards, members of the expert panel assessed each item in a two-round Delphi survey. Items that met agreement in the first or second round were included in the final version of the criteria. Results: Positive screening for SpA if at least one of the following is present: onset of chronic low back pain before 45 years of age; inflammatory low back pain or alternating buttock pain; HLA-B27 positivity; sacroiliitis on imaging; arthritis; heel enthesitis; dactylitis. Positive screening for IBD in the presence of one of the major criteria or at least two minor criteria. Major: rectal bleeding; chronic diarrhea with organic characteristics; perianal disease. Minor: chronic abdominal pain; iron deficiency anemia or iron deficiency; extraintestinal manifestations; fever or low grade fever, of unknown origin and duration >1week; unexplained weight loss; family history of IBD. Conclusion: Screening criteria for IBD in patients with SpA, and vice versa, have been developed. These criteria will be useful for early detection of both diseases (AU)

Screening of Inflammatory Bowel Disease and Spondyloarthritis for Referring Patients Between Rheumatology and Gastroenterology. / Criterios de cribado de enfermedad inflamatoria intestinal y espondiloartritis para derivación de pacientes entre Reumatología y Gastroenterología.

OBJECTIVE: To define clinical screening criteria for spondyloarthritis (SpA) in patients with inflammatory bowel disease (IBD) and vice versa, which can be used as a reference for referring them to the rheumatology or gastroenterology service. METHOD: Systematic literature review and a two-round Delphi method. The scientific committee and the expert panel were comprised of 2 rheumatologists and 2 gastroenterologists, and 7 rheumatologists and 7 gastroenterologists, respectively. The scientific committee defined the initial version of the criteria, taking into account sensitivity, specificity, standardization and ease of application. Afterwards, members of the expert panel assessed each item in a two-round Delphi survey. Items that met agreement in the first or second round were included in the final version of the criteria. RESULTS: Positive screening for SpA if at least one of the following is present: onset of chronic low back pain before 45 years of age; inflammatory low back pain or alternating buttock pain; HLA-B27 positivity; sacroiliitis on imaging; arthritis; heel enthesitis; dactylitis. Positive screening for IBD in the presence of one of the major criteria or at least two minor criteria. Major: rectal bleeding; chronic diarrhea with organic characteristics; perianal disease. Minor: chronic abdominal pain; iron deficiency anemia or iron deficiency; extraintestinal manifestations; fever or low grade fever, of unknown origin and duration >1week; unexplained weight loss; family history of IBD. CONCLUSION: Screening criteria for IBD in patients with SpA, and vice versa, have been developed. These criteria will be useful for early detection of both diseases.

Recommendations by the Spanish Society of Rheumatology on the Use of Biological Therapies in Axial Spondyloarthritis. / Recomendaciones de la Sociedad Española de Reumatología sobre el uso de terapias biológicas en espondiloartritis axial.

OBJECTIVE: Recent data published on biological therapy in axial spondyloarthritis (axSpA) since the last publication of the recommendations of the Spanish Society of Rheumatology (SER) has led to the generation of a review of these recommendations based on the best possible evidence. These recommendations should be a reference for rheumatologists and those involved in the treatment of patients with axSpA. METHODS: Recommendations were drawn up following a nominal group methodology and based on systematic reviews. The level of evidence and grade of recommendation were classified according to the model proposed by the Centre for Evidence Based Medicine at Oxford. The level of agreement was established through the Delphi technique. RESULTS: In this review, we did an update of the evaluation of disease activity and treatment objectives. We included the new drugs with approved therapeutic indication for axSpA. We reviewed both the predictive factors of the therapeutic response and progression of radiographic damage. Finally, we drafted some recommendations for the treatment of patients refractory to anti-tumor necrosis factor, as well as for the possible optimization of biological therapy. The document also includes a table of recommendations and a treatment algorithm. CONCLUSIONS: We present an update of the SER recommendations for the use of biological therapy in patients with axSpA.

Screening of Inflammatory Bowel Disease and Spondyloarthritis for Referring Patients Between Rheumatology and Gastroenterology. / Criterios de cribado de enfermedad inflamatoria intestinal y espondiloartritis para derivación de pacientes entre Reumatología y Gastroenterología.

OBJECTIVE: To define clinical screening criteria for spondyloarthritis (SpA) in patients with inflammatory bowel disease (IBD) and vice versa, which can be used as a reference for referring them to the rheumatology or gastroenterology service. METHOD: Systematic literature review and a two-round Delphi method. The scientific committee and the expert panel were comprised of 2 rheumatologists and 2 gastroenterologists, and 7 rheumatologists and 7 gastroenterologists, respectively. The scientific committee defined the initial version of the criteria, taking into account sensitivity, specificity, standardization and ease of application. Afterwards, members of the expert panel assessed each item in a two-round Delphi survey. Items that met agreement in the first or second round were included in the final version of the criteria. RESULTS: Positive screening for SpA if at least one of the following is present: onset of chronic low back pain before 45 years of age; inflammatory low back pain or alternating buttock pain; HLA-B27 positivity; sacroiliitis on imaging; arthritis; heel enthesitis; dactylitis. Positive screening for IBD in the presence of one of the major criteria or at least two minor criteria. Major: rectal bleeding; chronic diarrhea with organic characteristics; perianal disease. Minor: chronic abdominal pain; iron deficiency anemia or iron deficiency; extraintestinal manifestations; fever or low grade fever, of unknown origin and duration >1week; unexplained weight loss; family history of IBD. CONCLUSION: Screening criteria for IBD in patients with SpA, and vice versa, have been developed. These criteria will be useful for early detection of both diseases.

Clinical utility of the ASDAS index in comparison with BASDAI in patients with ankylosing spondylitis (Axis Study).

The objective of the study was to study the clinical utility of the Ankylosing Spondylitis Disease Activity Score (ASDAS) for the assessment of disease activity in ankylosing spondylitis (AS) patients, compared to the Bath Ankylosing Spondylitis Activity Index (BASDAI). This was a prospective longitudinal observational study in patients with AS (NY-modified criteria) from 23 Spanish centers. Physical and analytical data; global, lumbar, and nocturnal pain; ASDAS, BASDAI and minimally acceptable clinical status (PASS) were collected. Psychometric characteristics of both indexes were analyzed: construct validity (convergent and divergent), discriminant capacity, criterion validity (global physician and patient assessment), and sensitivity to change. The study involved 127 patients (19.7% attrition). Both BASDAI and ASDAS showed a higher correlation with patient's global assessment (r = 0.76 and 0.70, respectively) than with physician's global assessment (r = 0.67 and 0.57). Both scores allowed discriminating patients with an acceptable clinical status, although BASDAI to a greater extent than ASDAS (Cohen δ 1.72 vs 0.88 for the medical PASS). Both scores showed sensitivity to change in patients who changed from an unacceptable symptomatic state to acceptable according to PASS criteria (physician and patient) and by BASDAI 50 response criteria (Cohen δ > 0.80). BASDAI showed better criterion validity than ASDAS, both for the patient PASS (AUC 0.85 vs 0.79) and for the physician's (AUC 0.90 vs 0.79). ASDAS shows adequate performance for disease activity in patients with AS; however, in this study, its psychometric properties do not present advantages over BASDAI in terms of criterion validity, sensitivity to change or discriminative capacity; replacement of BASDAI by ASDAS is not supported by the data.

Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies.

The aim of this study was to assess nailfold capillaroscopic (NC) findings in patients with primary Sjögren's syndrome (PSS) with and without Raynaud's phenomenon (RP) as well as in the presence of positive anti-SSA/Ro and anti-SSB/La antibodies. Videocapillaroscopy was performed in 150 patients with PSS. Data collected included demographics, presence of RP, PSS symptoms, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, and NC findings. RP was present in 32% of PSS, keratoconjunctivitis sicca in 91%, oral xerosis in 93%, and skin or genital xerosis in 53%. In patients with positive anti-SSA/Ro (75%) and positive anti-SSB/La (40%), NC showed normal findings in 53% of cases and non-specific in 36%. In patients with PSS, NC was normal in 51% of cases and non-specific in 34%. Scleroderma pattern was found in 14 patients. RP associated with PSS had non-specific capillaroscopy in 40% of cases (p = 0.1). Pericapillary haemorrhages (p = 0.06) and capillary thrombosis (p = 0.2) were not increased, but more dilated capillaries were detected in 48% of cases. Patients with positive anti-Ro and/or anti-La have not a distinct NC profile. Patients with RP associated with PSS had more dilated capillaries, but neither pericapillary haemorrhages nor capillary thrombosis was observed.

Recomendaciones para la detección, investigación y derivación del dolor lumbar inflamatorio en Atención Primaria / Reccomendations for the detection, study and referral of inflammatory low-back pain in Primary Care

Objetivo. Diseñar una estrategia de detección y derivación precoz de pacientes con posible espondiloartritis mediante el desarrollo de recomendaciones consensuadas dirigidas a los médicos de Atención Primaria (AP). Métodos. Se utilizó una metodología modificada de RAND/UCLA y revisión sistemática de la literatura. Se seleccionó un grupo de discusión formado por reumatólogos y médicos de AP. Se estudió el mapa del proceso y se propusieron recomendaciones y algoritmos que fueron sometidos a 2 rondas Delphi para evaluar el grado de aceptación y preferencia de criterios en un grupo amplio de reumatólogos y médicos de AP. Del análisis de la segunda ronda Delphi se extrajeron las recomendaciones finales. Resultados. Se presentan recomendaciones, junto con su grado medio de acuerdo, para la derivación rápida de pacientes con sospecha de espondiloartritis. En concreto, se recomienda investigar el dolor lumbar crónico en menores de 45 años en 4 fases: 1) clínica: preguntas clave; 2) clínica: preguntas extra; 3) exploración física, y 4) pruebas complementarias. Se debe derivar a Reumatología si existen: 1) dolor lumbar inflamatorio; 2) signos indicativos de espondiloartritis, o 3) HLA B27 positivo, elevación de proteína C reactiva o signos radiológicos de sacroilitis. Se incluyen recomendaciones sobre el proceso de derivación y otras adicionales. Conclusiones. El grado de acuerdo con estas sencillas recomendaciones es amplio. Es necesario diseñar estrategias de formación y sensibilización desde los servicios de Reumatología para mantener una óptima colaboración de AP en la identificación de los casos y facilitar que los servicios de Reumatología estén preparados para asumir las derivaciones (AU)

Objective. To design a strategy for the early detection and referral of patients with possible spondyloarthritis based on recommendations developed, agreed upon, and directed to primary care physicians. Methods. We used a modified RAND/UCLA methodology plus a systematic literature review. The information was presented to a discussion group formed by rheumatologists and primary care physicians. The group studied the process map and proposed recommendations and algorithms that were subsequently submitted in two Delphi rounds to a larger group of rheumatologists and primary care physicians. The final set of recommendations was derived from the analysis of the second Delphi round. Results. We present the recommendations, along with their mean level of agreement, on the early referral of patients with possible spondyloarthritis. The panel recommends that the study of chronic low back pain in patients under 45 years be performed in four phases 1) clinical: key questions, 2) clinical: extra questions, 3) physical examination, and 4) additional tests. Conclusions. The level of agreement with these simple recommendations is high. It is necessary to design strategies for the education and sensitization from rheumatology services to maintain an optimal collaboration with primary care and to facilitate referral to rheumatology departments (AU)

Reccomendations for the detection, study and referral of inflammatory low-back pain in primary care.

OBJECTIVE: To design a strategy for the early detection and referral of patients with possible spondyloarthritis based on recommendations developed, agreed upon, and directed to primary care physicians. METHODS: We used a modified RAND/UCLA methodology plus a systematic literature review. The information was presented to a discussion group formed by rheumatologists and primary care physicians. The group studied the process map and proposed recommendations and algorithms that were subsequently submitted in two Delphi rounds to a larger group of rheumatologists and primary care physicians. The final set of recommendations was derived from the analysis of the second Delphi round. RESULTS: We present the recommendations, along with their mean level of agreement, on the early referral of patients with possible spondyloarthritis. The panel recommends that the study of chronic low back pain in patients under 45 years be performed in four phases 1) clinical: key questions, 2) clinical: extra questions, 3) physical examination, and 4) additional tests. CONCLUSIONS: The level of agreement with these simple recommendations is high. It is necessary to design strategies for the education and sensitization from rheumatology services to maintain an optimal collaboration with primary care and to facilitate referral to rheumatology departments.

Different clinical expression of patients with ankylosing spondylitis according to gender in relation to time since onset of disease. Data from REGISPONSER.

OBJECTIVE: To describe the differential characteristics by gender and time since disease onset in patients diagnosed with ankylosing spondylitis (AS) attending the Spanish rheumatology clinics, including those on the "Spanish Registry of spondyloarthritis" (REGISPONSER), as well as the diagnostic and therapeutic implications that this entails. PATIENTS AND METHODS: This is a transversal and observational study of 1514 patients with AS selected from 2367 spondyloarthritis cases included in REGISPONSER. For each patient, the demographics, epidemiology, geriatric, clinical, laboratory, radiological, and therapeutic aspects were were evaluated and comprehensively recorded under the aegis of REGISPONSER, constituting the Minimum Basic identifying data for the disease. Physical function was assessed by Bath Ankylosing Spondylitis Functional Index (BASFI). Clinical activity was evaluated using erythrocyte sedimentation rate, C reactive protein and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Each patient underwent pelvic anteroposterior, anteroposterior and lateral lumbar spine as well as lateral cervical spine x rays; they were scored according to the Bath Ankylosing Spondylitis Spine Radiographic Index, which measures structural damage. RESULTS: Of the 1514 patients screened, 1131 (74.7%) were men. We found significant differences in age at onset of symptoms as well as in the day of inclusion, between the two groups, being lower in men. We also obtained differences in the duration of the disease, which was lower in women. As for the existence of a history of AS among first-degree relatives, family forms were more common among women. The mean BASDAI score was also higher in women, regardless of time since onset of disease. In contrast, the improvement of pain with the use of NSAID's and radiological severity were higher in men, both reaching statistical significance. CONCLUSIONS: Among the Spanish AS patients, there are some differences in the clinical manifestations, even when the time since onset of disease was controlled; we also found radiological differences by gender; men showing more structural damage, while women were more active. These data suggest that the phenotype of AS differs between genders. This can influence the subsequent diagnostic approach and therapeutic decisions.

Genetic polymorphisms inside and outside the MHC improve prediction of AS radiographic severity in addition to clinical variables.

OBJECTIVE: The aim of this study was to analyse if single nucleotide polymorphisms (SNPs) inside and outside the MHC region might improve the prediction of radiographic severity in AS. METHODS: A cross-sectional multi-centre study was performed including 473 Spanish AS patients previously diagnosed with AS following the Modified New York Criteria and with at least 10 years of follow-up from the first symptoms of AS. Clinical variables and 384 SNPs were analysed to predict radiographic severity [BASRI-total (BASRI-t) corrected for the duration of AS since first symptoms] using multivariate forward logistic regression. Predictive power was measured by the area under the receiver operating characteristic curve (AUC), specificity, sensitivity, positive predictive value (PPV) and negative predictive value (NPV). RESULTS: The model with the best fit measured radiographic severity as the BASRI-t 60th percentile and combined eight variables: male gender, older age at disease onset and six SNPs at ADRB1 (rs1801253), NELL1 (rs8176785) and MHC (rs1634747, rs9270986, rs7451962 and rs241453) genes. The model predictive power was defined by AUC = 0.76 (95% CI 0.71, 0.80), being significantly better than the model with only clinical variables, AUC = 0.68 (95% CI 0.63, 0.73), P = 0.0004. Internal split-sample analysis proved the validation of the model. Patient genotype for SNPs outside the MHC region, inside the MHC region and clinical variables account for 26, 38 and 36%, respectively, of the explained variability on radiographic severity prediction. CONCLUSION: Prediction of radiographic severity in AS based on clinical variables can be significantly improved by including SNPs both inside and outside the MHC region.

[Consensus Statement of the Spanish Society of Rheumatology on the management of biologic therapies in spondyloarthritis except for psoriatic arthritis]. / Documento SER de consenso sobre el uso de terapias biológicas en la espondilitis anquilosante y otras espondiloartritis, excepto la artritis psoriásica.

OBJECTIVE: Due to the amount and variability in quality regarding the use of biologic therapy (BT) in patients with spondyloarthritis (SpA), except for psoriatic arthritis (PsA) patients, the Spanish Society of Rheumatology has promoted the generation of recommendations based on the best evidence available. These recommendations should be a reference for rheumatologists and those involved in the treatment of patients with spondyloarthritis (SpA), except for psoriatic arthritis (PsA), who are using, or about to use BT. METHODS: Recommendations were developed following a nominal group methodology and based on systematic reviews. The level of evidence and grade of recommendation were classified according to the model proposed by the Center for Evidence Based Medicine at Oxford. The level of agreement was established through Delphi technique. RESULTS: We have produced recommendations on the use of BT currently available for SpA (but not PsA) in our country. These recommendations include disease assessment, treatment objectives, therapeutic scheme and switching. CONCLUSIONS: We present an update on the SER recommendations for the use of BT in patients with SpA, except for PsA.

[Consensus statement of the Spanish Society of Rheumatology on the management of biologic therapies in psoriatic arthritis]. / Documento SER de consenso sobre el uso de terapias biológicas en la artritis psoriásica.

OBJECTIVE: Due to the amount and quality variability regarding the use of biologic therapy (BT) in psoriatic arthritis (PsA) patients, the Spanish Society of Rheumatology (SER) has promoted the generation of recommendations based on the best evidence available. These recommendations should serve as reference to rheumatologists and those involved in the treatment of patients with PsA, who are using, or about to use BT. METHODS: Recommendations were developed following a nominal group methodology and based on systematic reviews. The level of evidence and degree of recommendation was classified according to the model proposed by the Center for Evidence Based Medicine at Oxford. The level of agreement was established through Delphi technique. RESULTS: We have produced recommendations for the use of TB currently available for PsA in our country. These recommendations include disease assessment, treatment objectives, therapeutic scheme and switching. CONCLUSIONS: We present an update on the SER recommendations for the use of BT in patients with PsA.

Documento SER de consenso sobre el uso de terapias biológicas en la artritis psoriásica / Consensus statement of the Spanish Society of Rheumatology on the management of biologic therapies in psoriatic arthritis

Objetivo. Dada la gran cantidad de información actual sobre el uso de terapias biológicas (TB) en la artritis psoriásica (APs), y la variabilidad de la misma en cuanto a su calidad, desde la Sociedad Española de Reumatología (SER) se ha impulsado la generación de recomendaciones basadas en la mejor evidencia posible. Éstas deben de servir de referencia para reumatólogos e implicados en el tratamiento de APs que vayan a utilizar o consideren la utilización de TB. Métodos. Las recomendaciones se emitieron siguiendo la metodología de grupos nominales y basadas en revisiones sistemáticas. El nivel de evidencia y el grado de recomendación se clasificaron según el modelo del Center for Evidence Based Medicine de Oxford y el grado de acuerdo se extrajo por técnica Delphi. Resultados. Se realizan recomendaciones sobre el uso de las TB disponibles en la actualidad en nuestro país para el tratamiento de la APs. Estas recomendaciones incluyen la evaluación de la enfermedad, objetivos del tratamiento, esquema terapéutico y cambios en el mismo. Conclusiones. Se presentan las actualizaciones a las recomendaciones SER para el uso de TB en pacientes con APs (AU)

Objective. Due to the amount and quality variability regarding the use of biologic therapy (BT) in psoriatic arthritis (PsA) patients, the Spanish Society of Rheumatology (SER) has promoted the generation of recommendations based on the best evidence available. These recommendations should serve as reference to rheumatologists and those involved in the treatment of patients with PsA, who are using, or about to use BT. Methods. Recommendations were developed following a nominal group methodology and based on systematic reviews. The level of evidence and degree of recommendation was classified according to the model proposed by the Center for Evidence Based Medicine at Oxford. The level of agreement was established through Delphi technique. Results. We have produced recommendations for the use of TB currently available for PsA in our country. These recommendations include disease assessment, treatment objectives, therapeutic scheme and switching. Conclusions. We present an update on the SER recommendations for the use of BT in patients with PsA (AU)

Documento SER de consenso sobre el uso de terapias biológicas en la espondilitis anquilosante y otras espondiloartritis, excepto la artritis psoriásica / Consensus statement of the Spanish Society of Rheumatology on the management of biologic therapies in spondyloarthritis except for psoriatic arthritis

Objetivo. Dada la gran cantidad de información sobre las terapias biológicas (TB) en las espondiloartritis (EspA), excepto la artritis psoriásica (APs), y la variabilidad en cuanto a su calidad, desde la Sociedad Española de Reumatología (SER) se ha impulsado la generación de recomendaciones basadas en la mejor evidencia posible. Estas deben de servir de referencia para reumatólogos e implicados en el tratamiento de estos pacientes. Métodos. Las recomendaciones se emitieron siguiendo la metodología de grupos nominales. El nivel de evidencia y el grado de recomendación se clasificaron según el modelo del Center for Evidence Based Medicine de Oxford y el grado de acuerdo se extrajo por técnica Delphi. Resultados. Se realizan recomendaciones sobre el uso de las TB para el tratamiento de las EspA (excepto la APs). Incluyen la evaluación de la enfermedad, objetivos del tratamiento, esquema terapéutico y cambios en éste. Conclusiones. Se presentan las actualizaciones a las recomendaciones SER para el uso de TB en pacientes con EsA, excepto la APs(AU)

Objective. Due to the amount and variability in quality regarding the use of biologic therapy (BT) in patients with spondyloarthritis (SpA), except for psoriatic arthritis (PsA) patients, the Spanish Society of Rheumatology has promoted the generation of recommendations based on the best evidence available. These recommendations should be a reference for rheumatologists and those involved in the treatment of patients with spondyloarthritis (SpA), except for psoriatic arthritis (PsA), who are using, or about to use BT. Methods. Recommendations were developed following a nominal group methodology and based on systematic reviews. The level of evidence and grade of recommendation were classified according to the model proposed by the Center for Evidence Based Medicine at Oxford. The level of agreement was established through Delphi technique. Results. We have produced recommendations on the use of BT currently available for SpA (but not PsA) in our country. These recommendations include disease assessment, treatment objectives, therapeutic scheme and switching. Conclusions. We present an update on the SER recommendations for the use of BT in patients with SpA, except for PsA(AU)

Fármacos durante el embarazo y métodos contraceptivos en enfermedades reumáticas. Nuevas aportaciones / Drugs employed during pregnancy and contraceptive methods in rheumatic disease. New evidence

El control voluntario de la natalidad, el hecho de poder establecer el mejor momento de la gestación en función de la actividad de la enfermedad y la necesidad de evitar el embarazo debido a la indicación de fármacos potencialmente teratógenos constituyen las razones principales para la utilización de métodos contraceptivos en pacientes afectas de enfermedades reumáticas. En este artículo se revisan los posibles riesgos durante el embarazo de las medicaciones utilizadas en reumatología y los diversos métodos contraceptivos disponibles actualmente. En los últimos años se ha producido un mejor conocimiento de la seguridad de los métodos contraceptivos hormonales con datos especialmente relevantes de su utilización en pacientes con lupus eritematoso sistémico. La evolución de la contracepción hormonal con nuevas vías de administración como la transdérmica, la intravaginal y los dispositivos intrauterinos o los implantes liberadores de progestágenos han significado avances evidentes en este campo. La implicación del reumatólogo, como responsable de la atención de estos pacientes, es requerida con mayor frecuencia y el conocimiento de todos los métodos disponibles, con todas sus ventajas, desventajas y efectos adversos ayudará a facilitar información para que la pareja pueda escoger en cada caso la mejor opción(AU)

Voluntary birth control, the ability to identify the best moment for becoming pregnant depending on disease activity, and the need to avoid conception during the administration of teratogenic drugs are the main reasons for the use of contraceptive methods among women with rheumatic diseases. This article reviews the risks that antirheumatic drugs represent during conception, pregnancy and lactation and the contraceptive methods that are currently available to patients. Hormonal therapy has developed considerably and can further our understanding of safety aspects, especially for systemic lupus erythematosus patients. Recently the methods of administration have evolved, and now include transdermal and intravaginal routes, a progesterone-releasing intrauterine device, and an extended-cycle oral contraceptive. Rheumatologists work increasingly in conjunction with patients to assist in choices regarding contraceptive methods and pregnancy planning. Each decision should be individualized according to the personal preference and the stage of reproductive life(AU)

[Drugs employed during pregnancy and contraceptive methods in rheumatic disease. New evidence]. / Fármacos durante el embarazo y métodos contraceptivos en enfermedades reumáticas. Nuevas aportaciones.

Voluntary birth control, the ability to identify the best moment for becoming pregnant depending on disease activity, and the need to avoid conception during the administration of teratogenic drugs are the main reasons for the use of contraceptive methods among women with rheumatic diseases. This article reviews the risks that antirheumatic drugs represent during conception, pregnancy and lactation and the contraceptive methods that are currently available to patients. Hormonal therapy has developed considerably and can further our understanding of safety aspects, especially for systemic lupus erythematosus patients. Recently the methods of administration have evolved, and now include transdermal and intravaginal routes, a progesterone-releasing intrauterine device, and an extended-cycle oral contraceptive. Rheumatologists work increasingly in conjunction with patients to assist in choices regarding contraceptive methods and pregnancy planning. Each decision should be individualized according to the personal preference and the stage of reproductive life.

REGISPONSER II / No disponible

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