Search

Objective: To assess the real-world, long-term effectiveness of rituximab (RTX) as a rescue therapy in patients with antisynthetase syndrome and progressive interstitial lung disease (ASS-ILD). Methods: Multicentre observational retrospective longitudinal study of a cohort of patients with ASS-ILD that started treatment with RTX due to recurrent or ongoing progressive ILD despite therapy with glucocorticoids and immunosuppressants. Results: Twenty-eight patients were analyzed. Examining the entire study population, before treatment with RTX the mean decline in %pFVC and %pDLCO from the ASS-ILD diagnosis to the initiation of RTX treatment (T0) was -6.44% and -14.85%, respectively. After six months of treatment, RTX reversed the decline in pulmonary function test (PFT) parameters: ∆%pFVC +6.29% (95% CI: -10.07 to 2.51; p=0.002 compared to T0) and ∆%pDLCO +6.15% (95% CI: -10.86 to -1.43; p=0.013). Conclusion: Based on our results, RTX appears to be effective as rescue therapy in most patients with recurrent or progressive ASS-ILD unresponsive to conventional treatment. The use of RTX was well tolerated in the majority of patients.

Objective: To investigate the usefulness of 18F-FDG PET-CT for assessing large-vessel (LV) involvement in patients with suspected giant cell arteritis (GCA) and a negative temporal artery biopsy (TAB). Methods: A retrospective review of our hospital databases was conducted to identify patients with suspected GCA and negative TAB who underwent an 18F-FDG PET-CT in an attempt to confirm the diagnosis. The gold standard for GCA diagnosis was clinical confirmation after a follow-up period of at least 12 months. Results: Out of the 127 patients included in the study, 73 were diagnosed with GCA after a detailed review of their medical records. Conclusion: Our study confirms the utility of 18F-FDG PET-CT in patients presenting with suspected GCA and a negative TAB by demonstrating the presence of LV involvement across different subsets of the disease.

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).

ObjectiveTo investigate the prevalence, severity, and associated clinical factors of mitral and aortic valvular involvement in patients with systemic sclerosis (SSc).MethodsOur case-control study included 172 patients with SSc and 172 non-SSc adults without known cardiac disease matched by age, sex, and prevalence of cardiovascular (CV) risk factors. The screening of mitral and aortic valvular involvement was performed by transthoracic Doppler echocardiogram. The prevalence of aortic stenosis (AS) was also compared with that reported in a population-based study performed in our community during the same period.ResultsPatients with SSc showed an almost 5-fold increased prevalence of moderate to severe mitroaortic valve dysfunction compared to non-SSc controls (OR 4.60, 95% CI 1.51–13.98; P= 0.003). The most common lesion was mitral regurgitation (MR), which was observed in 5.2% of patients, followed by AS in 3.5%, and aortic regurgitation (AR) in 1.7%. Analyzing the different types of valvular lesion separately, we observed a significantly higher frequency of MR compared to controls (OR 4.69, 95% CI 1.12–22.04; P= 0.032), as well as a higher frequency of AS in the 65–75 (OR 7.51, 95% CI 1.22–46.23, P= 0.01) and 76–85 age groups (OR 3.53, 95% CI 1.03–12.22, P= 0.043) when compared to the general population in our community.ConclusionWe found an increased prevalence of moderate to severe MR and AS in SSc compared to age-matched non-SSc controls with similar CV comorbidities. While results from this study do not allow for establishing a direct causal relationship, they strongly support the contribution of SSc-specific factors in the development of these complications.

El lupus eritematoso sistémico (LES) es una enfermedad autoinmunitaria multisistémica compleja, con una gran heterogeneidad en su presentación clínica y una alta morbimortalidad. Aunque su pronóstico ha mejorado de forma notable a lo largo de las últimas décadas, aún siguen existiendo necesidades no cubiertas en esta enfermedad. En esta actualización realizamos una puesta al día de los principales avances en esta enfermedad en los últimos años. Concretamente revisamos los nuevos criterios de clasificación propuestos por la Liga Europea de Reumatología y el Colegio Americano de Reumatología, la búsqueda de nuevos biomarcadores, las nuevas definiciones de remisión y de baja actividad de la enfermedad, así como las estrategias actuales de abordaje y tratamiento del LES y la situación actual de las nuevas terapias.

Analizar las características clínicas, tratamiento y evolución de la vasculitis reumatoide.

Analizar el riesgo de fractura calculado por FRAX® y la frecuencia de alto riesgo de fractura en la población general en España.

To estimate the incidence and analyze any cancer‐associated factors in patients with systemic lupus erythematosus (SLE), differentiating between hormone‐sensitive (HS) and non‐HScancers. This was a retrospective multicenter study of a patient cohort from the Systemic Lupus Erythematosus Registry of the Spanish Society of Rheumatology. Included were the first cancer post‐SLEdiagnosis, clinical and sociodemographic information, cumulative damage, severity, comorbidities, treatments, and refractoriness. Cancers were classified as HS(prostate, breast, endometrium, and ovarian) and non‐HS(the remainder). The standardized incidence ratio (SIR) was calculated and logistic regression models were built. A total of 3,539 patients (90.4% women) were included, 154 of whom had cancer (91% female), and 44 had HScancer (100% female). The cancer SIR was 1.37 (95% confidence interval [95% CI] 1.15–1.59), with higher values in women age <65 years (SIR 2.38 [95% CI1.84–2.91]). The SIR in women with HSversus non‐HScancer was 1.02 (95% CI0.13–1.91) and 1.93 (95% CI0.98–2.89). In HSversus non‐HScancers, SLEdiagnostic age (odds ratio [OR] 1.04 [P= 0.002] versus 1.04 [P= 0.019]), and period of disease evolution (OR1.01 [P< 0.001] versus 1.00 [P= 0.029]) were associated with cancer. The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (OR1.27 [P= 0.022]) and angiotensin‐converting enzyme (ACE) inhibitor prescriptions (OR2.87 [P= 0.048]) were associated with non‐HScancers. Cancer incidence in patients with SLEwas higher than in the Spanish population, particularly among young women. This increase might be due to non‐HScancers, which would be associated with SLEinvolving greater cumulative damage where more ACEinhibitors are prescribed.

Objective.To describe the incidence of bacteremia in a large multicentric cohort of patients with systemic lupus erythematosus (SLE) and their clinical characteristics and to identify risk factors.Methods.All bacteremic episodes from the Spanish RELESSER registry were included. Clinical and laboratory characteristics concerning bacteremia and SLE status, as well as comorbidities at the time of infection, were retrospectively collected. A comparison with sex- and age-matched SLE controls without bacteremia was made. A logistic regression was conducted.Results.The study included 114 episodes of bacteremia in 83 patients. The incidence rate was 2.7/1000 patient-years. At the time of bacteremia, the median age was 40.5 (range: 8–90) years, and 88.6% of patients were female. The Safety of Estrogens in Lupus Erythematosus National Assessment–Systemic Lupus Erythematosus Disease Activity Index was 4 [interquartile range (IQR) 8]; 41% had an SLE flare (66% severe); Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was 3 (IQR 4). A comorbidity was recorded in 64% of cases. At the time of bacteremia, 88.6% received corticosteroids (68.6% > 10 mg/day) and 57% immunosuppressors. Gram-negative bacilli, most frequently Escherichia coli(29.8%), caused 52.6% of the episodes. The bacteremia-related mortality was 14% and bacteremia was recurrent in 27.2% of cases. A dose-response relationship was found between corticosteroids and bacteremia risk. In the multivariate analysis, these factors were associated with bacteremia: elevated creatinine (OR 1.31, 95% CI 1.01–1.70; p = 0.045), diabetes (OR 6.01, 95% CI 2.26–15.95; p < 0.001), cancer (OR 5.32, 95% CI 2.23–12.70; p < 0.001), immunosuppressors (OR 6.35, 95% CI 3.42–11.77; p < 0.001), and damage (OR 1.65, 95% CI 1.31–2.09; p < 0.001).Conclusion.Bacteremia occurred mostly in patients with active SLE and was frequently associated with severe flares and corticosteroid use. Recurrence and mortality were high. Immunosuppressors, comorbidities, and disease-related damage were associated with bacteremia.

Para llegar al diagnóstico de arteritis de células gigantes (ACG) se evalúan signos, síntomas, pruebas de laboratorio, hallazgos de pruebas de imagen y, en ocasiones, los resultados anatomopatológicos de la biopsia de arteria temporal (BAT). El presente estudio describe los resultados del análisis de un algoritmo basado en la clínica y la ecografía de los pacientes con sospecha de ACG, destacando su utilidad diagnóstica, al contrastar su utilización en diferentes escenarios de sospecha clínica.

The purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort. All adult patients in the RELESSER-TRANS (Registry of Systemic Lupus Erythematosus Patients of the Spanish Society of Rheumatology [SER], cross-sectional phase) registry were retrospectively investigated for the presence of primary pleuropulmonary manifestations. In total 3215 patients were included. At least one pleuropulmonary manifestation was present in 31% of patients. The most common manifestation was pleural disease (21%), followed by lupus pneumonitis (3.6%), pulmonary thromboembolism (2.9%), primary pulmonary hypertension (2.4%), diffuse interstitial lung disease (2%), alveolar hemorrhage (0.8%), and shrinking lung syndrome (0.8%). In the multivariable analysis, the variables associated with the development of pleuropulmonary manifestation were older age at disease onset (odds ratio (OR) 1.03, 95% confidence interval (CI) 1.02–1.04), higher SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) scores (OR 1.03, 95% CI 1.00–1.07), the presence of Raynaud’s phenomenon (OR 1.41, 95% CI 1.09–1.84), secondary antiphospholipid syndrome (OR 2.20, 95% CI 1.63–2.97), and the previous or concomitant occurrence of severe lupus nephritis, (OR 1.48, 95% CI 1.12–1.95) neuropsychiatric manifestations (OR 1.49, 95% CI 1.11–2.02), non-ischemic cardiac disease (OR 2.91, 95% CI 1.90–4.15), vasculitis (OR 1.81, 95% CI 1.25–2.62), hematological manifestations (OR 1.31, 95% CI 1.00–1.71), and gastrointestinal manifestations, excluding hepatitis (OR 2.05, 95% CI 1.14–3.66). Anti-RNP positivity had a clear tendency to significance (OR 1.32, 95% CI 1.00–1.75; P= 0.054). The development of pleuropulmonary manifestations independently contributes to a diminished survival (hazard ratio of 3.13). However, not all complications will influence the prognosis in the same way. Whereas the occurrence of pleural disease or pulmonary thromboembolism has a minimal impact on the survival of these patients, the remaining manifestations have a major impact on mortality. Except for pleural disease, the remaining respiratory manifestations are very uncommon in SLE (<4%). Pleuropulmonary manifestations independently contributed to a decreased survival in these patients.

La enfermedad de Still del adulto es una entidad infrecuente a medio camino entre los síndromes autoinflamatorios y las enfermedades autoinmunes, con una gran heterogeneidad en su presentación clínica, gravedad y evolución. En este artículo se revisan las novedades y aspectos menos conocidos de esta enfermedad referidos a su espectro clínico (lesiones cutáneas atípicas, complicaciones distintas de las manifestaciones clásicas, síndrome de activación macrofágica, fenotipos de la enfermedad), a la controversia existente acerca de su posible asociación con neoplasias, la búsqueda de biomarcadores para su diagnóstico, la evaluación del pronóstico (factores clínicos, índices pronósticos y biomarcadores) y los factores determinantes en la elección del tratamiento biológico.

Evaluar la efectividad de una intervención sobre los factores de riesgo cardiovascular (FRCV) en pacientes con artritis reumatoide.

Background: Visual involvement is the most feared complication of giant cell arteritis (GCA). Information on the efficacy of tocilizumab (TCZ) for this complication is scarce and controversial.Objective: We assessed a wide series of GCA treated with TCZ, to evaluate its role in the prevention of new visual complications and its efficacy when this manifestation was already present before the initiation of TCZ.Design: This is an observational multicenter study of patients with GCA treated with TCZ.Methods: Patients were divided into two subgroups according to the presence or absence of visual involvement before TCZ onset. Visual manifestations were classified into the following categories: transient visual loss (TVL), permanent visual loss (PVL), diplopia, and blurred vision.Results: Four hundred seventy-one GCA patients (mean age, 74 ± 9 years) were treated with TCZ. Visual manifestations were observed in 122 cases (26%), of which 81 were present at TCZ onset: PVL (n= 60; unilateral/bilateral: 48/12), TVL (n= 17; unilateral/bilateral: 11/6), diplopia (n= 2), and blurred vision (n= 2). None of the patients without previous visual involvement or with TVL had new episodes after initiation of TCZ, while only 11 out of 60 (18%) patients with PVL experienced some improvement. The two patients with diplopia and one of the two patients with blurred vision improved.Conclusion: TCZ may have a protective effect against the development of visual complications or new episodes of TVL in GCA. However, once PVL was established, only a few patients improved.

We have hypothesized that incompatibility between the G1m genotype of the patient and the G1m1 and G1m17 allotypes carried by infliximab (INX) and adalimumab (ADM) could decrease the efficacy of these anti-tumor necrosis factor (anti-TNF) antibodies in the treatment of rheumatoid arthritis (RA). The G1m genotypes were analyzed in three collections of patients with RA totaling 1037 subjects. The first, used for discovery, comprised 215 Spanish patients. The second and third were successively used for replication. They included 429 British and Greek patients and 393 Spanish and British patients, respectively. Two outcomes were considered: change in the Disease Activity Score in 28 joint (ΔDAS28) and the European League Against Rheumatism (EULAR) response criteria. An association between less response to INX and incompatibility of the G1m1,17 allotype was found in the discovery collection at 6 months of treatment (P= 0.03). This association was confirmed in the replications (P= 0.02 and 0.08, respectively) leading to a global association (P= 0.001) that involved a mean difference in ΔDAS28 of 0.4 units between compatible and incompatible patients (2.3 ± 1.5 in compatible patients vs.1.9 ± 1.5 in incompatible patients) and an increase in responders and decrease in non-responders according to the EULAR criteria (P= 0.03). A similar association was suggested for patients treated with ADM in the discovery collection, but it was not supported by replication. Our results suggest that G1m1,17 allotypes are associated with response to INX and could aid improved therapeutic targeting in RA.

We aimed to assess a functional polymorphism in FCGR2AH131R, for association with the treatment response to Fc-containing inhibitors of tumor necrosis factor (TNF).

Objective.We evaluated whether a single-nucleotide polymorphism (SNP) of the TRAF6gene previously associated with systemic lupus erythematosus and rheumatoid arthritis may be a common risk factor for systemic sclerosis (SSc) and giant cell arteritis (GCA).Methods.A total of 1185 patients with SSc, 479 patients with biopsy-proven GCA, and 1442 unrelated healthy controls of white Spanish origin were genotyped for the rs540386 variant using a specifically designed TaqMan©allele discrimination assay.Results.No significant associations of this SNP with global SSc or GCA were found. This was also the case when the potential associations of the TRAF6polymorphism with the main clinical phenotypes of the 2 diseases (e.g., limited cutaneous and diffuse cutaneous SSc, or presence of polymyalgia rheumatica and visual ischemic manifestations in GCA) were assessed.Conclusion.Our data do not support a role of the rs540386 TRAF6variant as a key component of the genetic network underlying SSc and GCA.

OBJECTIVE: To investigate whether a functional integrin alpha M (ITGAM) variant is involved in susceptibility to and clinical manifestations of giant cell arteritis (GCA). METHODS: A Spanish cohort of 437 white patients with biopsy-proven GCA and 1388 healthy controls were genotyped using the TaqMan allele discrimination technology. RESULTS: No association was observed between ITGAM rs1143679 and GCA (p = 0.80, OR 0.97). Similarly, subphenotype analyses did not yield significant differences between the case subgroups and the control set or between GCA patients with or without the main specific features of GCA. CONCLUSION: Our results suggest that the ITGAM rs1143679 variant does not play an important role in the pathophysiology of GCA.

OBJECTIVE: To assess the potential association between the rs1343151 IL23R and the rs3790567 IL12RB2 polymorphisms and giant cell arteritis (GCA). We also studied whether these polymorphisms might influence the phenotypic expression of GCA. METHODS: In total, 357 Spanish patients with biopsy-proven GCA and 574 matched controls were assessed. DNA from patients and controls was obtained from peripheral blood. Samples were genotyped for the rs1343151 IL23R and the rs3790567 IL12RB2 polymorphisms using a predesigned TaqMan allele discrimination assay and by polymerase chain reaction amplification. RESULTS: Regarding the rs1343151 IL23R polymorphism, no significant differences in the genotype or allele frequencies between GCA patients and healthy controls were observed. The frequency of the minor allele A of the rs3790567 IL12RB2 variant was increased in GCA patients compared with controls (30.1% vs 25.7%, respectively; p = 0.039, OR 1.25, 95% CI 1.01–1.54). An increased frequency of subjects carrying the minor allele A (GA+AA genotypes) of the rs3790567 IL12RB2 polymorphism was found among GCA patients compared with controls (52.8% vs 44.4%; p = 0.013, OR 1.40, 95% CI 1.06–1.85). Although a higher frequency of the combination of minor alleles (A-A) in the subgroup of patients with visual ischemic complications compared with the combination of both major alleles (G-G; p = 0.029) or with the other allelic combinations (p = 0.035) was found, logistic regression analysis showed that this association was no longer significant after adjustment for potential confounding factors (A-A vs G-G: OR 2.10, 95% CI 0.88–5.04, p = 0.096). CONCLUSION: Our results support a potential influence of the rs3790567 IL12RB2 polymorphism in the pathogenesis of GCA.

No abstract.

OBJECTIVE: Although several decision rules have been developed to identify postmenopausal women who may be selected for dual-energy x-ray absorptiometry measurements, information on their utility in a clinical setting is scarce. We evaluated the utility of 4 previously validated decision rules in a large group of Spanish postmenopausal women referred to a bone densitometry unit. METHODS: We reviewed the data on 665 postmenopausal women (mean age 54.2 ± 5.4 yrs). We selected the 4 decision rules that could be applied with the information that was available: the Osteoporosis Risk Assessment Instrument (ORAI), Osteoporosis Self-Assessment Tool (OST), Osteoporosis Index of Risk (OSIRIS), and Body Weight Criterion (BWC). The sensitivity, specificity, and predictive values of each decision rule were determined. RESULTS: The ORAI would recommend 45% of women for bone mineral density (BMD) testing, OST 46%, OSIRIS 37%, and BWC 70%. Sensitivity values obtained in the overall series were 64.1% for the ORAI, 69.2% for OST, 58.1% for OSIRIS, and 83.8% for BWC. The sensitivity increased progressively with age. The negative predictive value in the overall series was 88.5% for ORAI, 89.9% for OST, 88.4% for OSIRIS, and 90.6% for BWC. CONCLUSION: In a complementary way with previous studies in older women, where decision rules were valuable to identify the majority of women likely to have osteoporosis, our data indicate that in younger postmenopausal women, decision rules are useful as a screening method to rule out the presence of osteoporosis and the need for BMD scanning.

OBJECTIVE: To address a controversy regarding the existence of a relative adrenal hypofunction in patients with untreated polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) we evaluated baseline serum levels of ACTH, cortisol, and DHEAS in a cohort of patients with recent onset PMR/GCA not previously treated with glucocorticoids, in comparison with healthy controls. Possible correlations between baseline DHEAS levels and laboratory measures of disease activity were also explored. METHODS: Basal serum levels of these hormones were prospectively investigated in 25 patients with active untreated disease and compared with those of 25 age- and sex-matched control subjects. RESULTS: Of the 25 patients, 19 had isolated PMR and 6 had biopsy-proven GCA + PMR. Basal levels of cortisol and ACTH in PMR/GCA patients did not differ from control subjects; in relation to inflammatory status, lower than expected basal production of cortisol was observed in active untreated PMR/GCA. Baseline serum DHEAS levels were significantly lower in all patients compared with controls. In these patients, a significant correlation was found between baseline DHEAS values and laboratory measures of disease activity. The percentage of DHEAS reduction and the severity of inflammatory response were higher in women than in men. CONCLUSION: Patients with PMR/GCA with new-onset active disease before steroid treatment have inappropriately normal cortisol levels regarding the ongoing inflammation, and significantly lower levels of DHEAS compared to the age- and sex-matched healthy control subjects. These data support the existence of a relative adrenal hypofunction in PMR and GCA.

No abstract.

OBJECTIVE: An association between chronic hepatitis C virus (HCV) infection and fibromyalgia (FM) remains controversial, mainly because previous studies were based on prevalent case series or comparisons with less than optimal control groups. We investigated whether there might be an association between chronic HCV infection and FM. METHODS: We prospectively investigated the prevalence of HCV infection in a series of 115 patients with FM and compared it with the prevalence in the general population of our community reported in the same period. Anti-HCV antibodies were determined by ELISA. In positive cases, infection was confirmed by recombinant immunoblot assay and HCV-RNA was detected by PCR using sera samples. Differences between prevalence rates were assessed by chi-square test. RESULTS: HCV infection was confirmed in 3 of 115 patients with FM (2.6%). Two of these patients (1.74%) had active HCV infection shown by the presence of viral RNA in serum, whereas HCV RNA was undetectable in the third patient. In these cases, liver disease had previously been undiagnosed and HCV infection manifested itself by extrahepatic symptoms. Although the prevalence of HCV infection was slightly higher in patients with FM than in the general population in the age groups 25– 44 and 45– 64 years, when we compared prevalence rates in the total group and the different age groups, no statistically significant differences were found. CONCLUSION: From our results, it seems unlikely that HCV infection plays a pathogenic role in FM.

Objective: To assess the efficacy and safety of tocilizumab (TCZ) in Caucasian patients with refractory Takayasu’s arteritis (TAK) in clinical practice.Methods: A multicenter study of Caucasian patients with refractory TAK who received TCZ. The outcome variables were remission, glucocorticoid-sparing effect, improvement in imaging techniques, and adverse events. A comparative study between patients who received TCZ as monotherapy (TCZMONO) and combined with conventional disease modifying anti-rheumatic drugs (cDMARDs) (TCZCOMBO) was performed.Results: The study comprised 54 patients (46 women/8 men) with a median [interquartile range (IQR)] age of 42.0 (32.5–50.5) years. TCZ was started after a median (IQR) of 12.0 (3.0–31.5) months since TAK diagnosis. Remission was achieved in 12/54 (22.2%), 19/49 (38.8%), 23/44 (52.3%), and 27/36 (75%) patients at 1, 3, 6, and 12 months, respectively. The prednisone dose was reduced from 30.0 mg/day (12.5–50.0) to 5.0 (0.0–5.6) mg/day at 12 months. An improvement in imaging findings was reported in 28 (73.7%) patients after a median (IQR) of 9.0 (6.0–14.0) months. Twenty-three (42.6%) patients were on TCZMONOand 31 (57.4%) on TCZCOMBO: MTX (n= 28), cyclosporine A (n= 2), azathioprine (n= 1). Patients on TCZCOMBOwere younger [38.0 (27.0–46.0) versus45.0 (38.0–57.0)] years; difference (diff) [95% confidence interval (CI) = -7.0 (-17.9, -0.56] with a trend to longer TAK duration [21.0 (6.0–38.0) versus6.0 (1.0–23.0)] months; diff 95% CI = 15 (-8.9, 35.5), and higher c-reactive protein [2.4 (0.7–5.6) versus1.3 (0.3–3.3)] mg/dl; diff 95% CI = 1.1 (-0.26, 2.99). Despite these differences, similar outcomes were observed in both groups (log rank p= 0.862). Relevant adverse events were reported in six (11.1%) patients, but only three developed severe events that required TCZ withdrawal.Conclusion: TCZ in monotherapy, or combined with cDMARDs, is effective and safe in patients with refractory TAK of Caucasian origin.

Abstract:  We report a case of MFH of the mandible in a 16-year-old girl. The mandible is a rare location of MFH of bone, with only 21 cases described in the literature. Clinical, pathologic, and radiologic features are discussed, with special emphasis on the unusual pathologic and radiographic features.

CD226 genetic variants have been associated with a number of autoimmune diseases and recently with systemic sclerosis (SSc). The aim of this study was to test the influence of CD226 loci in SSc susceptibility, clinical phenotypes and autoantibody status in a large multicenter European population.

The article presents a study which evaluates the cost-effectiveness of the injectable inactivated polio vaccine (IPV) introduced in Colombia by comparing it to the existing oral polio vaccine (OPV).