Práctica clínica en la arteritis de células gigantes a partir de una encuesta a especialistas / Clinical practice in giant cell arteritis based on a survey of specialists

Antecedentes: El propósito de este estudio fue conocer la práctica clínica de especialistas que atienden a pacientes con arteritis de células gigantes, para comprobar si siguen las recomendaciones para el diagnóstico y tratamiento de esta enfermedad e identificar áreas de mejora. Métodos: Encuesta transversal de prácticas clínicas realizada en 2019. Ciento sesenta y siete médicos (64% reumatólogos, 27% especialistas en medicina interna, 9% otros especialistas) que asistieron a un curso de actualización del tratamiento de la arteritis de células gigantes completó la encuesta. Comparamos la práctica clínica recogida en el estudio con las últimas recomendaciones aprobadas por la Liga Europea Contra el Reumatismo (EULAR). Resultados: Los médicos encuestados atendían a una mediana de 10 pacientes (rango intercuartílico 6-30) con arteritis de células gigantes en su práctica clínica. Como método de diagnóstico, los encuestados utilizaron biopsia de arteria temporal (84%), ecografía de arteria temporal (61%) u otras técnicas de imagen (37%). Como terapia de primera línea, los encuestados utilizaron glucocorticoides en dosis altas (al menos 40mg de prednisona o equivalente por día) (84%), glucocorticoides con metotrexato (7%) y glucocorticoides con tocilizumab (5%). Los fármacos más utilizados para la recaída fueron el metotrexato (37%) y tocilizumab (58%). Conclusión: Los resultados de esta encuesta indican que los médicos especialistas encuestados siguen las recomendaciones recientes de EULAR sobre diagnóstico y tratamiento de la arteritis de células gigantes (AU)

Background: The purpose of this study was to learn about the clinical practice of specialists who care for patients with giant cell arteritis, to verify whether they follow the diagnosis and treatment recommendations for this disease, and to identify areas for improvement. Methods: A cross-sectional survey on clinical practice in 2019. The survey was completed by 167 physicians (64% rheumatologists, 27% internal medicine specialists, and 9% other specialists) who attended a course on updating giant cell arteritis treatment. We compared the clinical practice collected in the study with the latest recommendations approved by the European League Against Rheumatism (EULAR). Results: The physicians surveyed cared for a median of 10 patients (interquartile range 6-30) with giant cell arteritis during their practice. As a diagnostic method, respondents used temporal artery biopsy (84%), temporal artery ultrasound (61%) or other imaging techniques (37%). As first-line therapy, respondents used high-dose glucocorticoids (at least 40mg of prednisone, or equivalent, per day) (84%), glucocorticoids with methotrexate (7%) and glucocorticoids with tocilizumab (5%). The most frequent drugs used for relapse were methotrexate (37%) and tocilizumab (58%). Conclusion: Our results indicate that the medical specialists surveyed follow the recent EULAR recommendations for giant cell arteritis diagnosis and therapy (AU)

Clinical practice in giant cell arteritis based on a survey of specialists.

BACKGROUND: The purpose of this study was to learn about the clinical practice of specialists who care for patients with giant cell arteritis, to verify whether they follow the diagnosis and treatment recommendations for this disease, and to identify areas for improvement. METHODS: A cross-sectional survey on clinical practice in 2019. The survey was completed by 167 physicians (64% rheumatologists, 27% internal medicine specialists, and 9% other specialists) who attended a course on updating giant cell arteritis treatment. We compared the clinical practice collected in the study with the latest recommendations approved by the European League Against Rheumatism (EULAR). RESULTS: The physicians surveyed cared for a median of 10 patients (interquartile range 6-30) with giant cell arteritis during their practice. As a diagnostic method, respondents used temporal artery biopsy (84%), temporal artery ultrasound (61%) or other imaging techniques (37%). As first-line therapy, respondents used high-dose glucocorticoids (at least 40 mg of prednisone, or equivalent, per day) (84%), glucocorticoids with methotrexate (7%) and glucocorticoids with tocilizumab (5%). The most frequent drugs used for relapse were methotrexate (37%) and tocilizumab (58%). CONCLUSION: Our results indicate that the medical specialists surveyed follow the recent EULAR recommendations for giant cell arteritis diagnosis and therapy.

Necrotizing Sarcoid Granulomatosis: A Disease Not to be Forgotten.

Sarcoidosis is a systemic granulomatous disease of unknown aetiology characterised by the appearance of noncaseifying epithelioid granulomas in the affected organs, most commonly the lungs, skin, and eyes (Iannuzzi et al. 2007). Necrotizing Sarcoid Granulomatosis (NGS) is a rare and little-known form of disease, which also presents nodular lung lesions, and it shares pathologic and clinical findings with sarcoidosis, where the presence of necrosis may lead to misdiagnosis of tuberculosis (TB), leading to a consequent delay in treatment of the underlying entity (Chong et al. 2015). This is exactly what happened with the two cases that we present here.

Diet Quality and High-Sensitivity C-Reactive Protein in Patients With Systemic Lupus Erythematosus.

BACKGROUND AND AIMS: C-reactive protein (CRP) is commonly used as a biomarker for inflammation. Mild elevations of CRP have been seen in chronic autoimmune diseases like systemic lupus erythematosus (SLE), and CRP has been linked to an increased risk of cardiovascular events. Diet quality and certain dietary factors seem to influence CRP levels in healthy subjects. To date, the effect of diet on serum CRP in SLE has not been studied. Our aim was to investigate the relationship between dietary nutrients, antioxidant intake, and serum CRP in SLE. DESIGN AND METHOD: A cross-sectional study was conducted among 91 patients with SLE. High-sensitivity hsCRP values were determined using an immuno-turbidimetry assay in a Beckman Coulter analyzer (AU5800). Dietary intake of macro- and micronutrients was assessed through a 24-hr diet recall. Antioxidant nutrient intake was evaluated using the dietary antioxidant quality score (DAQs). Linear regression models were used to investigate the relationships between serum hsCRP levels, dietary nutrient intake, and DAQs. RESULTS: The mean serum hsCRP level observed (3.76 ± 6.68 mg/L) was above the established normal range. However, participating SLE patients had low-quality diets, and we found no significant correlations between dietary intake of macro- or micronutrients or antioxidant nutrient intake (DAQs) and serum CRP levels. CONCLUSION: Our study reveals that participating SLE patients had a low-quality diet that did not influence inflammatory status measured using serum CRP levels. Further interventional studies with high-quality diets in this population are necessary to dissect the role of diet on CRP levels in SLE.

Combined mepacrine-hydroxychloroquine treatment in patients with systemic lupus erythematosus and refractory cutaneous and articular activity.

Aim The aim of this study was to evaluate the clinical response to combined therapy with hydroxychloroquine and mepacrine in patients with systemic lupus erythematosus and refractory joint and/or skin disease. Methods Mepacrine was added to 46 systemic lupus erythematosus patients unresponsive to treatment with the following drug combinations: hydroxychloroquine + prednisone + immunosuppressive drugs ( n = 24), hydroxychloroquine + prednisone ( n = 16), hydroxychloroquine + prednisone + retinoids ( n = 2), hydroxychloroquine alone ( n = 1), hydroxychloroquine + one immunosuppressive drug ( n = 1), hydroxychloroquine + prednisone + one immunosuppressive drug + belimumab ( n = 1) or hydroxychloroquine + prednisone + belimumab ( n = 1). The outcome variable was the clinical response, either complete or partial, based on clinical judgement. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score were additionally used. Results A total of 91% patients showed complete/partial response, with similar rates among those with joint or skin disease. In patients with cutaneous activity, a statistically significant decrease in the CLASI was seen. There also was a statistically significant decrease in the SLEDAI. The mean daily dose of prednisone decreased from 5.8 to 3.4 mg/d ( p = 0.001). Prednisone could be discontinued in 20% of patients. No serious adverse events were seen. Smoking was the only predictor of complete response. Conclusion In the setting of refractory skin and/or joint disease, the addition of mepacrine to previous therapy including hydroxychloroquine was safe and effective in reducing disease activity and decreasing prednisone doses. The fact that smokers responded better opens the door to further studying the combination of mepacrine-hydroxychloroquine as a first-line therapy in such patients.

Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.

OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group. RESULTS: Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD. CONCLUSIONS: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).

First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study.

The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud's phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud's phenomenon may be considered of poor prognosis.

Enfermedades autoinflamatorias en el adulto. Características clínicas e implicaciones pronósticas / Autoinflammatory diseases in adults. Clinical characteristics and prognostic implications

Las enfermedades autoinflamatorias son cuadros clínicos con manifestaciones inflamatorias que se presentan de forma periódica o persistente, producidas por alteraciones adquiridas o hereditarias de la respuesta inmune innata. En general, son más frecuentes en la edad pediátrica, pero se han descrito casos en adultos, por lo que son de interés para cualquier especialista. Existen pocas referencias de estas enfermedades en el adulto por su baja prevalencia e infradiagnóstico. El objetivo de este trabajo es revisar la literatura científica sobre estos trastornos para sistematizar sus características clínicas, pronósticas y la respuesta al tratamiento en el adulto (AU)

Autoinflammatory diseases are clinical conditions with inflammatory manifestations that present in a periodic or persistent manner and are caused by acquired or hereditary disorders of the innate immune response. In general, these diseases are more common in childhood, but cases have been reported in adults and are therefore important for all specialists. There are few references on these diseases in adults due to their low prevalence and underdiagnosis. The aim of this study is to review the scientific literature on these disorders to systematise their clinical, prognostic and treatment response characteristics in adults (AU)

Autoinflammatory diseases in adults. Clinical characteristics and prognostic implications. / Enfermedades autoinflamatorias en el adulto. Características clínicas e implicaciones pronósticas.

Autoinflammatory diseases are clinical conditions with inflammatory manifestations that present in a periodic or persistent manner and are caused by acquired or hereditary disorders of the innate immune response. In general, these diseases are more common in childhood, but cases have been reported in adults and are therefore important for all specialists. There are few references on these diseases in adults due to their low prevalence and underdiagnosis. The aim of this study is to review the scientific literature on these disorders to systematise their clinical, prognostic and treatment response characteristics in adults.

The effects of corticosteroids on cognitive flexibility and decision-making in women with lupus.

The aim of this study was to investigate the possible effects of corticosteroids in women with systemic lupus erythematosus (SLE) in two processes of executive function: cognitive flexibility and decision-making. To that end, we evaluated 121 women divided into three groups: 50 healthy women, 38 women with SLE not receiving corticosteroid treatment and 33 women with SLE receiving corticosteroid treatment. Cognitive flexibility was measured with the Trail Making Tests A and B; decision-making was measured with the Iowa Gambling Task. Additionally, demographic (age and education level), clinical (SLE Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI) and disease duration) and psychological characteristics (stress vulnerability, perceived stress and psychopathic symptomatology) were evaluated. The results showed that both SLE groups displayed poorer decision-making than the healthy women ( p = 0.006) and also that the SLE group receiving corticosteroid treatment showed lower cognitive flexibility than the other two groups ( p = 0.030). Moreover, SLE patients showed poorer scores than healthy women on the following SCL-90-R subscales: somatisation ( p = 0.005), obsessions and compulsions ( p = 0.045), depression ( p = 0.004), hostility ( p = 0.013), phobic anxiety ( p = 0.005), psychoticism ( p = 0.016) and positive symptom total ( p = 0.001). In addition, both SLE groups were more vulnerable to stress ( p = 0.000). These findings help to understand the effects of corticosteroid treatment on cognitive flexibility and decision-making, in addition to the disease-specific effects suffered by women with SLE.

Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.

Antiphospholipid syndrome, antiphospholipid antibodies and solid organ transplantation.

Antiphospholipid syndrome is considered a high risk factor for any kind of surgery. Considering that all solid organ transplants are critically dependent on the patency of vascular anastomosis, there is much concern about the consequences this pro-thrombotic condition may have on transplantation. Relatively little information is available in the literature assessing the real risk that antiphospholipid syndrome or the presence of antiphospholipid antibodies represent in solid organ transplantation. The aim of this article is to review the literature related to transplantation of solid organs in patients diagnosed with antiphospholipid syndrome or patients with positive antiphospholipid antibodies.

The role of antiphospholipid autoantibodies in the cognitive deficits of patients with systemic lupus erythematosus.

OBJECTIVE: To analyze the role of the antiphospholipid autoantibodies (aPL) on the neuropsychological deficits in systemic lupus erythematosus (SLE) patients, comparing groups of patients with antiphospholipid syndrome (APS; n = 15), SLE with aPL (n = 12), and SLE without aPL (n = 27), and a healthy control group (n = 31). METHODS: Patients fulfilled the American College of Rheumatology SLE classification criteria or the Sydney criteria for APS. All participants were woman, and groups were matched on age and education. A standardized cognitive examination classified patients as cognitively declined or impaired according to the American College of Rheumatology. RESULTS: Differences between the groups were found in all of the studied variables, comprising attention and executive functions (sustained and selective attention, fluency, and inhibition), and memory (verbal and visual). Post-hoc analyses showed cognitive performance was equivalent between APS and SLE with aPL. Differences between SLE without aPL and control groups were found only in four of the 10 studied variables, while differences in all but two memory variables were found between SLE without aPL and control groups. Furthermore, cognitive deficit was three times more frequent in APS and SLE with aPL patients than for the control group (80%, 75%, and 16%, respectively), and two times more frequent compared to SLE patients without aPL (48%). CONCLUSIONS: Our results support the relationship between aPL and cognitive symptoms in SLE. Also, almost half of the patients with SLE and no aPL showed cognitive problems, pointing to the multifactorial causes of cognitive problems in SLE. Future research with larger sample size is guaranteed to replicate our results.

Pyoderma gangrenosum and systemic lupus erythematosus: a report of five cases and review of the literature.

Pyoderma gangrenosum (PG) is an uncommon, distinctive cutaneous ulceration which is usually idiopathic, but may be associated with many systemic disorders. The etiopathogenesis of PG is still not well understood. PG is part of the spectrum of the neutrophilic dermatoses and it has been proposed as a prototype of cutaneous autoinflammatory disease. PG usually has a good outcome under immunosuppressive treatment. Although PG has been associated with several systemic diseases, it has rarely been reported in association with systemic lupus erythematosus (SLE). In this article we report five cases of SLE-related PG and review the literature. Our findings support the possible relationship between active SLE and PG, although the mechanism remains unclear. Clinical manifestations, used treatments and outcomes of SLE-related PG do not differ from the described for the general population.

Influence of the IL17A locus in giant cell arteritis susceptibility.

OBJECTIVE: Different lines of evidence have highlighted the role of IL-17A in the inflammatory process occurring in giant cell arteritis (GCA). The aim of the present study was to assess whether the IL17A locus influences GCA susceptibility and its clinical subphenotypes. METHODS: We carried out a large meta-analysis including a total of 1266 biopsy-proven GCA patients and 3779 healthy controls from four European populations (Spain, Italy, Germany and Norway). Five IL17A polymorphisms (rs4711998, rs8193036, rs3819024, rs2275913 and rs7747909) were selected by tagging and genotyped using TaqMan assays. Allelic combination and dependency tests were also performed. RESULTS: In the pooled analysis, two of the five analysed polymorphisms showed evidence of association with GCA (rs2275913: PMH=1.85E-03, OR=1.17 (1.06-1.29); rs7747909: PMH=8.49E-03, OR=1.15 (1.04-1.27)). A clear trend of association was also found for the rs4711998 variant (PMH=0.059, OR=1.11 (1.00-1.23)). An independent effect of rs2275913 and rs4711998 was evident by conditional regression analysis. In addition, the haplotype harbouring the risk alleles better explained the observed association than the polymorphisms independently (likelihood p value <10(-05)). CONCLUSIONS: Polymorphisms within the IL17A locus show a novel association with GCA. This finding supports the relevant role of the Th17 cells in this vasculitis pathophysiology.