Valor de las herramientas clínicas de predicción de osteoporosis en la artritis reumatoide. Sensibilidad, especificidad y valores predictivos en pacientes atendidos en dos servicios de Reumatología según práctica clínica habitual

En el ámbito de la artritis reumatoide se diseñaron herramientas específicas para seleccionar a que pacientes se debería hacer una densitometría ósea. Apenas existe información sobre su aplicación en la práctica asistencial y no se ha analizado si aportan valor diferencial respecto a las herramientas diseñadas para regular la realización de una densitometría en la población general. La práctica universal del estudio densitométrico no es viable y desde hace años se insiste en la necesidad de establecer una selección de los pacientes tributarios a la realización de esta técnica. En este estudio analizamos la sensibilidad, la especificidad, el valor predictivo positivo y el valor predictivo negativo de diversas herramientas de predicción de osteoporosis en artritis reumatoide precoz. Se incluyeron 298 pacientes. Los criterios de Edimburgo mostraron adecuada sensibilidad y apropiado valor predictivo negativo. Mostraron tambien un comportamiento homogéneo en ambos géneros, hombres y mujeres y resultaron aplicables a pacientes que recibian tratamiento glucocorticoide

Sarcopenia, immune-mediated rheumatic diseases, and nutritional interventions

Introduction: Sarcopenia is defined by a loss of muscle mass and function associated with mortality, decreased physical performance, falls, and disability. Since chronic inflammation and decreased physical activity are risk factors for developing sarcopenia, it is critical to assess the role of sarcopenia in immune-mediated rheumatic diseases (IMRDs). Moreover, nutritional interventions are emerging as key modifiable and affordable options to improve physical performance in sarcopenia. Objective: The aim of this review is to critically summarize current information on the evidence linking nutritional interventions and sarcopenia in IMRDs. Methods: The search and selection of articles was performed in Medline, Dimensions.ai, Google Scholar, Cochrane Library, Epistemonikos, and Trip Database. The results were clustered into three areas: sarcopenia and IMRDs, sarcopenia and biological disease-modifying antirheumatic drugs (bDMARDs), and nutritional interventions for sarcopenia. Findings: Several cross-sectional studies have shown a higher prevalence of sarcopenia in IMRDs, such as rheumatoid arthritis. Although not fully established, evidence linking sarcopenia and other IMRDs (ankylosing spondylitis and systemic sclerosis) has been also described. For secondary sarcopenia prevention and treatment, bDMARDs' administration proved efficacy in patients with rheumatoid arthritis. Furthermore, there is growing evidence linking nutrition to the prevention and treatment of sarcopenia. Evidence linking unfavourable results in nutritional risk assessment, insufficient intake of protein, vitamin D, antioxidant nutrients, and long-chain polyunsaturated fatty acids and sarcopenia have been reported. Conclusion: Given that sarcopenia and IMRDs have strong links, further research is needed to improve patient care

Wells’ Syndrome Successfully Treated with Colchicine

La celulitis eosinofílica es un trastorno infrecuente, inflamatorio y crónico de etiología desconocida. Actualmente, se considera que los corticosteroides son el tratamiento de primera línea, pero no carecen de desventajas significativas, como las contraindicaciones en los casos resistentes a esteroides y los pacientes con recidivas frecuentes. Divulgamos a un paciente que sufre del síndrome de Wells con una historia de 24 años de lesiones cutáneas sintomáticas y generalizadas. Después de la consulta en nuestro servicio, se prescribió tratamiento con colchicina 1 mg / día, lo que resultó en una gran mejoría clínica. No se han registrado efectos secundarios. Hasta donde sabemos, este es un enfoque de enfermedad original. Aunque pequeña, nuestra experiencia clínica respalda la inclusión de colchicina en el arsenal farmacológico cuando se trata a pacientes con síndrome de Wells. De hecho, su excelente perfil de seguridad lo hace muy atractivo para los pacientes con episodios recurrentes frecuentes que necesitan opciones seguras para el control de la enfermedad a mediano y largo plazoA celulitis eosinofílica é un trastorno infrecuente, inflamatorio e crónico de etioloxía descoñecida. Actualmente, considérase que os corticosteroides son o tratamento de primeira liña, pero non carecen de desvantaxes significativas, como as contraindicacións nos casos resistentes a esteroides e os pacientes con recidivas frecuentes. Divulgamos a un paciente que sofre da síndrome de Wells cunha historia de 24 anos de lesións cutáneas sintomáticas e xeneralizadas. Despois da consulta no noso servizo, prescribiuse tratamento con colchicina 1 mg / día, o que resultou nunha gran melloría clínica. Non se rexistraron efectos secundarios. Ata onde sabemos, este é un enfoque da enfermidade orixinal. Aínda que pequena, a nosa experiencia clínica apoia a inclusión de colchicina no arsenal farmacolóxico cando se trata a pacientes con síndrome de Wells. De feito, o seu excelente perfil de seguridade faio moi atractivo para os pacientes con episodios recorrentes frecuentes que necesitan opcións seguras para o control da enfermidade a mediano e longo prazoEosinophilic cellulitis is an uncommon, inflammatory and chronic disorder of unknown etiology. Corticosteroids are currently considered as the first-line treatment but they are not without significant disadvantages such as contraindications in steroid-resistant cases and patients with frequent recurrences. We report a patient suffering from Wells’ syndrome with a 24-year history of symptomatic and generalized skin lesions. After consultation in our department, treatment with colchicine 1 mg/day was prescribed resulting in large clinical improvement. No side effects have been recorded. To our knowledge, this is an original disease approach. Although small, our clinical experience supports the inclusion of colchicine in the drug armamentarium when treating patients suffering from Wells’ syndrome. Indeed, its excellent safety profile makes it very attractive for patients with frequent recurrent episodes who need secure options for the medium- and long-term disease contro

Sarcoidosis información para pacientes y familiares

En portada aparecen logos de Ministerio de Sanidad, Servicios Sociales e Igualdad, Guiasalud.es, Red Española de Agencias de Evaluación de Tecnologías Sanitarias y Prestaciones del SNS

Evaluación de las necesidades y elaboración de un documento de información para pacientes con sarcoidosis basado en la evidencia

En portada aparecen logos de Ministerio de Sanidad, Servicios Sociales e Igualdad y Red Española de Agencias de Evaluación de Tecnologías y Prestaciones del SN

Effectiveness of janus kinase inhibitors in relapsing giant cell arteritis in real-world clinical practice and review of the literature

Background A substantial proportion of patients with giant cell arteritis (GCA) relapse despite standard therapy with glucocorticoids, methotrexate and tocilizumab. The Janus kinase/signal transducer and activator of transcription (JAK/STAT) signalling pathway is involved in the pathogenesis of GCA and JAK inhibitors (JAKi) could be a therapeutic alternative. We evaluated the effectiveness of JAKi in relapsing GCA patients in a real-world setting and reviewed available literature.Methods Retrospective analysis of GCA patients treated with JAKi for relapsing disease at thirteen centers in Spain and one center in United States (01/2017-12/2022). Outcomes assessed included clinical remission, complete remission and safety. Clinical remission was defined as the absence of GCA signs and symptoms regardless of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values. Complete remission was defined as the absence of GCA signs and symptoms along with normal ESR and CRP values. A systematic literature search for other JAKi-treated GCA cases was conducted.Results Thirty-five patients (86% females, mean age 72.3) with relapsing GCA received JAKi therapy (baricitinib, n = 15; tofacitinib, n = 10; upadacitinib, n = 10). Before JAKi therapy, 22 (63%) patients had received conventional synthetic immunosuppressants (e.g., methotrexate), and 30 (86%) biologics (e.g., tocilizumab). After a median (IQR) follow-up of 11 (6-15.5) months, 20 (57%) patients achieved and maintained clinical remission, 16 (46%) patients achieved and maintained complete remission, and 15 (43%) patients discontinued the initial JAKi due to relapse (n = 11 [31%]) or serious adverse events (n = 4 [11%]). A literature search identified another 36 JAKi-treated GCA cases with clinical improvement reported for the majority of them.Conclusions This real-world analysis and literature review suggest that JAKi could be effective in GCA, including in patients failing established glucocorticoid-sparing therapies such as tocilizumab and methotrexate. A phase III randomized controlled trial of upadacitinib is currently ongoing (ClinicalTrials.gov ID NCT03725202)

Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice

OBJECTIVE: Tocilizumab (TCZ) has shown efficacy in clinical trials on giant cell arteritis (GCA). Real-world data are scarce. Our objective was to assess efficacy and safety of TCZ in unselected patients with GCA in clinical practice Methods: Observational, open-label multicenter study from 40 national referral centers of GCA patients treated with TCZ due to inefficacy or adverse events of previous therapy. Outcomes variables were improvement of clinical features, acute phase reactants, glucocorticoid-sparing effect, prolonged remission and relapses. A comparative study was performed: (a) TCZ route (SC vs. IV); (b) GCA duration (?6 vs. >6 months); (c) serious infections (with or without); (d) ?15 vs. >15 mg/day at TCZ onset. RESULTS: 134 patients; mean age, 73.0 ± 8.8 years. TCZ was started after a median [IQR] time from GCA diagnosis of 13.5 [5.0-33.5] months. Ninety-eight (73.1%) patients had received immunosuppressive agents. After 1 month of TCZ 93.9% experienced clinical improvement. Reduction of CRP from 1.7 [0.4-3.2] to 0.11 [0.05-0.5] mg/dL (p < 0.0001), ESR from 33 [14.5-61] to 6 [2-12] mm/1st hour (p < 0.0001) and decrease in patients with anemia from 16.4% to 3.8% (p < 0.0001) were observed. Regardless of administration route or disease duration, clinical improvement leading to remission at 6, 12, 18, 24 months was observed in 55.5%, 70.4%, 69.2% and 90% of patients. Most relevant adverse side-effect was serious infections (10.6/100 patients-year), associated with higher doses of prednisone during the first three months of therapy. CONCLUSION: In clinical practice, TCZ yields a rapid and maintained improvement of refractory GCA. Serious infections appear to be higher than in clinical trials

Cranial and extracranial large-vessel giant cell arteritis share a genetic pattern of interferon-gamma pathway

OBJECTIVES: Two main different clinical phenotypes of giant cell arteritis (GCA) have been described, the classic cranial pattern and the extracranial large-vessel (LV) pattern. Since interferon gamma (IFNG) has shown to be a pivotal cytokine in the pathophysiology of GCA, our aim was to evaluate for the first time the influence of IFNG and IFNG receptor 1 (IFNGR1) polymorphisms in the different clinical phenotypes of GCA. METHODS: Two IFNG polymorphisms (rs2069718 G/A and rs1861493 A/G) and one polymorphism in IFNGR1 (rs1327474 G/A) were genotyped in 191 patients with biopsy-proven cranial GCA, 109 with extracranial LV-GCA and 490 healthy controls. A comparative study was conducted between patients with cranial and extracranial LV-GCA. RESULTS: No significant differences in genotype, allele, and haplotype frequencies of IFNG polymorphisms were found between GCA patients with the classic cranial pattern and the extracranial LV-GCA pattern. Similar results were found for genotype and allele frequencies of IFNGR1 polymorphism. It was also the case when patients with extracranial LV-GCA were compared with healthy controls. CONCLUSIONS: Our results show that IFNG and IFNGR1 polymorphisms do not influence the clinical phenotype of expression of GCA. Classic cranial GCA and extracranial LV-GCA seem to share a genetic pattern of IFNG pathway

Cranial and extracranial giant cell arteritis do not exhibit differences in the IL6 -174 G/C gene polymorphism

Since interleukin-6 (IL-6) is a pivotal proinflammatory cytokine implicated in the pathogenesis of giant cell arteritis (GCA), we aimed to determine the potential association of the functional IL6 -174 G/C polymorphism with GCA as well as if the single base change variation at the promoter region in the human IL-6 gene may account for differences in the clinical spectrum of GCA between cranial and extracranial large vessel vasculitis (LVV)-GCA

The presence of both HLA-DRB1[*]04:01 and HLA-B[*]15:01 increases the susceptibility to cranial and extracranial giant cell arteritis.

Objectives: To determine if patients with the predominant extracranial large-vessel-vasculitis (LVV) pattern of giant cell arteritis (GCA) have a distinctive HLA-B association, different from that reported in biopsy-proven cranial GCA patients. In a further step we assessed if the combination of HLA-B and HLA-DRB1 alleles confers an increased risk for GCA susceptibility, either for the cranial and extracranial LVV phenotypes. Methods: A total of 184 patients with biopsy-proven cranial GCA, 105 with LVV-GCA and 486 healthy controls were included in our study. We compared HLA-B phenotype frequencies between the three groups. Results: HLA-B*15 phenotype was significantly increased in patients with classic cranial GCA compared to controls (14.7% versus 5.8%, respectively; p<0.01; OR [95% CI] =2.81 [1.54-5.11]). It was mainly due to the HLA-B*15:01 allele (12.5% versus 4.0%, respectively; p<0.01; OR [95% CI] =3.51 [1.77-6.99]) and remained statistically significant after Bonferroni correction. Similar HLA-B*15 association was observed in patients with the LVV-GCA (11.4% versus 5.8%, p=0.04, OR [95% CI] =2.11 [1.04-4.30]). This association was also mainly due to the HLA-B*15:01 allele (10.5% versus 4.0%, respectively; p=0.0054; OR [95% CI] =2.88 [1.19-6.59]). Noteworthy, the presence of HLA-B*15:01 together with HLA-DRB1*04:01 led to an increased risk of developing both cranial and extracranial LVV-GCA. Conclusions: Susceptibility to GCA is strongly related to the HLA region, regardless of the clinical phenotype of expression of the disease.This work was partially supported by RETICS Programs, RD08/0075 (RIER), RD12/0009/0013 and RD16/0012 from ‘‘Instituto de Salud Carlos III’’ (ISCIII) (Spain). However, this research did not receive any specific grant from funding agencies in the commercial or not-for-profit sectors