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INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE. METHODS: Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed. RESULTS: 4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died. CONCLUSIONS: DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome.
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RATIONALE: Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis. PATIENT CONCERNS: A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern. DIAGNOSIS: Biliary cholangitis-like and digital ischemia related to cGVHD. INTERVENTIONS: The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome. OUTCOMES: After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function. LESSONS: cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association.
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Síndrome de Bronquiolite Obliterante , Colangite , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Escleroderma Sistêmico , Humanos , Feminino , Pessoa de Meia-Idade , Transplante Homólogo/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Doença Enxerto-Hospedeiro/complicações , Doença Enxerto-Hospedeiro/diagnóstico , Colangite/complicações , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/terapia , Isquemia/complicações , Doença CrônicaRESUMO
INTRODUCTION AND OBJECTIVES: Despite the evidence, there are doubts about the positioning of apremilast in the psoriatic arthritis (PsA) treatment algorithm. The objective of this project was to collect the scientific evidence and the experience of a group of rheumatologists who are experts in the management of PsA with apremilast in clinical practice in Spain. MATERIAL AND METHODS: A scientific committee made up of 6 experts proposed 5 clinical scenarios where the evidence on the use of apremilast in PsA was controversial: (i) Efficacy in peripheral PsA; (ii) Efficacy in enthesitis and dactylitis; (iii) Efficacy in PsA with skin involvement; (iv) Comorbidities; and (v) Apremilast safety. After this, a panel of 17 rheumatologists with expertise in PsA management discussed these scenarios and generated a questionnaire with 50 questions and 156 items following the Delphi methodology. This questionnaire was anonymously answered by the panel. RESULTS: After 2 voting rounds, the panel of experts reached consensus in 93 of the 156 items raised (59.6%) (67 in agreement and 26 in disagreement). The degree of consensus was 53.3% in the area of "Efficacy in peripheral PsA"; 60.0% in "Efficacy in enthesitis and dactylitis"; 50.0% in "Efficacy in PsA with skin involvement"; 57.1% in "Management of comorbidities in patients with PsA"; and 67.3% in "Implications of safety in the use of apremilast". CONCLUSIONS: The structured opinion of the experts complements the available evidence and contributes to the establishment of consensual guidelines for the use of apremilast in PsA.
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Artrite Psoriásica , Humanos , Artrite Psoriásica/tratamento farmacológico , Talidomida/uso terapêutico , Algoritmos , EspanhaRESUMO
OBJECTIVES: To analyze the prevalence, incidence, survival and contribution on mortality of major central nervous system (CNS) involvement in systemic lupus erythematosus (SLE). METHODS: Patients fulfilling the SLE 1997 ACR classification criteria from the multicentre, retrospective RELESSER-TRANS (Spanish Society of Rheumatology Lupus Register) were included. Prevalence, incidence and survival rates of major CNS neuropsychiatric (NP)-SLE as a group and the individual NP manifestations cerebrovascular disease (CVD), seizure, psychosis, organic brain syndrome and transverse myelitis were calculated. Furthermore, the contribution of these manifestations on mortality was analysed in Cox regression models adjusted for confounders. RESULTS: A total of 3591 SLE patients were included. Of them, 412 (11.5%) developed a total of 522 major CNS NP-SLE manifestations. 61 patients (12%) with major CNS NP-SLE died. The annual mortality rate for patients with and without ever major CNS NP-SLE was 10.8% vs 3.8%, respectively. Individually, CVD (14%) and organic brain syndrome (15.5%) showed the highest mortality rates. The 10% mortality rate for patients with and without ever major CNS NP-SLE was reached after 12.3â¯vs 22.8 years, respectively. CVD (9.8 years) and organic brain syndrome (7.1 years) reached the 10% mortality rate earlier than other major CNS NP-SLE manifestations. Major CNS NP-SLE (HR 1.85, 1.29-2.67) and more specifically CVD (HR 2.17, 1.41-3.33) and organic brain syndrome (HR 2.11, 1.19-3.74) accounted as independent prognostic factors for poor survival. CONCLUSION: The presentation of major CNS NP-SLE during the disease course contributes to a higher mortality, which may differ depending on the individual NP manifestation. CVD and organic brain syndrome are associated with the highest mortality rates.
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Lúpus Eritematoso Sistêmico , Vasculite Associada ao Lúpus do Sistema Nervoso Central , Reumatologia , Humanos , Estudos Retrospectivos , Lúpus Eritematoso Sistêmico/epidemiologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/complicações , Vasculite Associada ao Lúpus do Sistema Nervoso Central/epidemiologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/psicologia , Sistema Nervoso CentralRESUMO
OBJECTIVE: To compare the efficacy of infliximab (IFX) versus adalimumab (ADA) as a first-line biologic drug over 1 year of treatment in a large series of patients with refractory uveitis due to Behçet's disease (BD). METHODS: We conducted an open-label multicenter study of IFX versus ADA for BD-related uveitis refractory to conventional nonbiologic treatment. IFX or ADA was chosen as the first-line biologic agent based on physician and patient agreement. Patients received 3-5 mg/kg intravenous IFX at 0, 2, and 6 weeks and every 4-8 weeks thereafter, or 40 mg subcutaneous ADA every other week without a loading dose. Ocular parameters were compared between the 2 groups. RESULTS: The study included 177 patients (316 affected eyes), of whom 103 received IFX and 74 received ADA. There were no significant baseline differences between treatment groups in main demographic features, previous therapy, or ocular sign severity. After 1 year of therapy, we observed an improvement in all ocular parameters in both groups. However, patients receiving ADA had significantly better outcomes in some parameters, including improvement in anterior chamber inflammation (92.31% versus 78.18% for IFX; P = 0.06), improvement in vitritis (93.33% versus 78.95% for IFX; P = 0.04), and best-corrected visual acuity (mean ± SD 0.81 ± 0.26 versus 0.67 ± 0.34 for IFX; P = 0.001). A nonsignificant difference was seen for macular thickness (mean ± SD 250.62 ± 36.85 for ADA versus 264.89 ± 59.74 for IFX; P = 0.15), and improvement in retinal vasculitis was similar between the 2 groups (95% for ADA versus 97% for IFX; P = 0.28). The drug retention rate was higher in the ADA group (95.24% versus 84.95% for IFX; P = 0.042). CONCLUSION: Although both IFX and ADA are efficacious in refractory BD-related uveitis, ADA appears to be associated with better outcomes than IFX after 1 year of follow-up.
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Adalimumab/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Imunossupressores/uso terapêutico , Infliximab/uso terapêutico , Uveíte/tratamento farmacológico , Adulto , Síndrome de Behçet/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Uveíte/etiologiaRESUMO
OBJECTIVE: The main purpose of this recommendation statement is to provide clinicians with the best available evidence and the best opinion agreed upon by the panelists for a rational use of synthetic disease modifying antirheumatic drugs (DMARDs) and biologicals in psoriatic arthritis (PsA) patients. The present document also focuses on important aspects in the management of PsA, such as early diagnosis, therapeutic objectives, comorbidities and optimization of treatment. METHODS: The recommendations were agreed by consensus by a panel of 8 expert rheumatologists, previously selected by the Spanish Society of Rheumatology (SER) through an open call. The phases of the work were: identification of key areas for updating the previous consensus, analysis and synthesis of scientific evidence (modified Oxford system, Centre for Evidence-based Medicine, 2009) and formulation of recommendations based on this evidence and by consensus techniques. RESULTS: Seventeen recommendations were issued for the treatment of PsA patients. Six of them were of general nature, ranging from the early diagnosis and treatment to the importance of assessing comorbidities. The other 11 were focused on the indications for DMARDs and biological therapy in the distinct clinical forms of the disease. Likewise, the situation of failure of the first biological is addressed and treatment algorithms and a table with the different biological therapies are also included. CONCLUSIONS: We present the update of SER recommendations for the treatment of PsA with DMARDs and biologics.
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Antirreumáticos/uso terapêutico , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/diagnóstico , Terapia Biológica , Monitoramento de Medicamentos , Diagnóstico Precoce , Humanos , Espanha , Resultado do TratamentoRESUMO
Introducción y objetivos: A pesar de la evidencia, existen dudas sobre el posicionamiento de apremilast en el algoritmo de tratamiento de la artritis psoriásica (APs). El objetivo del presente proyecto fue recoger la evidencia científica y la experiencia de un grupo de reumatólogos expertos en el manejo de la APs sobre el uso de apremilast en la práctica clínica en España. Material y métodos: Un comité científico formado por 6 expertos propuso 5 escenarios clínicos donde la evidencia sobre el uso de apremilast en APs era controvertida: 1) eficacia en APs periférica; 2) eficacia en entesitis y dactilitis; 3) eficacia en APs con afectación cutánea; 4) comorbilidades, y 5) seguridad de apremilast. Tras esto, un panel de 17 reumatólogos expertos en el tratamiento de la APs discutió estos escenarios y generó un cuestionario con 50 preguntas y 156 ítems según metodología Delphi, el cual fue respondido de forma anónima por los panelistas. Resultados: Tras 2 rondas de votación, el panel de expertos alcanzó el consenso en 93 de los 156 ítems planteados (59,6%) (67 apropiados y 26 inapropiados). El grado de consenso fue del 53,3% en el área de «Eficacia en APs periférica»; del 60,0% en «Eficacia en entesitis y dactilitis»; del 50,0% en «Eficacia en APs con afectación cutánea»; del 57,1% en «Manejo de las comorbilidades en pacientes con APs», y del 67,3% en «Implicaciones de la seguridad en el uso de apremilast». Conclusiones: La opinión estructurada de los expertos complementa la evidencia disponible y contribuye al establecimiento de pautas consensuadas para el uso de apremilast en APs.(AU)
Introduction and objectives: Despite the evidence, there are doubts about the positioning of apremilast in the psoriatic arthritis (PsA) treatment algorithm. The objective of this project was to collect the scientific evidence and the experience of a group of rheumatologists who are experts in the management of PsA with apremilast in clinical practice in Spain. Material and methods: A scientific committee made up of 6 experts proposed 5 clinical scenarios where the evidence on the use of apremilast in PsA was controversial: 1) efficacy in peripheral PsA; 2) efficacy in enthesitis and dactylitis; 3) efficacy in PsA with skin involvement; 4) comorbidities, and 5) apremilast safety. After this, a panel of 17 rheumatologists with expertise in PsA management discussed these scenarios and generated a questionnaire with 50 questions and 156 items following the Delphi methodology. This questionnaire was anonymously answered by the panel. Results: After 2 voting rounds, the panel of experts reached consensus in 93 of the 156 items raised (59.6%) (67 appropiate and 26 inappropiate). The degree of consensus was 53.3% in the area of Efficacy in peripheral PsA; 60.0% in Efficacy in enthesitis and dactylitis; 50.0% in Efficacy in PsA with skin involvement; 57.1% in Management of comorbidities in patients with PsA, and 67.3% in Implications of safety in the use of apremilast. Conclusions: The structured opinion of the experts complements the available evidence and contributes to the establishment of consensual guidelines for the use of apremilast in PsA.(AU)
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Humanos , Masculino , Feminino , Artrite Psoriásica/tratamento farmacológico , Prova Pericial , Comorbidade , Consenso , Prática Clínica Baseada em Evidências , Reumatologia , Doenças ReumáticasRESUMO
The aim of the study was to profile those patients included in the RELESSER registry with histologically proven renal involvement in order to better understand the current state of lupus nephritis (LN) in Spain. RELESSER-TRANS is a multicenter cross-sectional registry with an analytical component. Information was collected from the medical records of patients with systemic lupus erythematosus who were followed at participating rheumatology units. A total of 359 variables including demographic data, clinical manifestations, disease activity, severity, comorbidities, LN outcome, treatments, and mortality were recorded. Only patients with a histological confirmation of LN were included. We performed a descriptive analysis, chi-square or Student's t tests according to the type of variable and its relationship with LN. Odds ratio and confidence intervals were calculated by using simple logistic regression. LN was histologically confirmed in 1092/3575 patients (30.5%). Most patients were female (85.7%), Caucasian (90.2%), and the mean age at LN diagnosis was 28.4â±â12.7 years. The risk for LN development was higher in men (M/F:47.85/30.91%, Pâ<â0.001), in younger individuals (Pâ<â0.001), and in Hispanics (Pâ=â0.03). Complete response to treatment was achieved in 68.3% of patients; 10.35% developed ESRD, which required a kidney transplant in 45% of such cases. The older the patient, the greater was the likelihood of complete response (Pâ<â0.001). Recurrences were associated with persistent lupus activity at the time of the last visit (Pâ<â0.001) and with ESRD (Pâ<â0.001). Thrombotic microangiopathy was a risk factor for ESRD (Pâ=â0.04), as for the necessity of dialysis (Pâ=â0.01) or renal transplantation (Pâ=â0.03). LN itself was a poor prognostic risk factor of mortality (OR 2.4 [1.81-3.22], Pâ<â0.001). Patients receiving antimalarials had a significantly lower risk of developing LN (Pâ<â0.001) and ESRD (Pâ<â0.001), and responded better to specific treatments for LN (Pâ=â0.014). More than two-thirds of the patients with LN from a wide European cohort achieved a complete response to treatment. The presence of positive anti-Sm antibodies was associated with a higher frequency of LN and a decreased rate of complete response to treatment. The use of antimalarials reduced both the risk of developing renal disease and its severity, and contributed to attaining a complete renal response.
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Nefrite Lúpica/epidemiologia , Sistema de Registros , Adolescente , Adulto , Feminino , Humanos , Nefrite Lúpica/terapia , Masculino , Recidiva , Estudos Retrospectivos , Reumatologia , Espanha/epidemiologia , Adulto JovemRESUMO
SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted.
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Síndrome de Hiperostose Adquirida/complicações , Veia Subclávia , Veia Cava Superior , Trombose Venosa/etiologia , Humanos , Fatores de RiscoRESUMO
Objetivo: La primera finalidad de este documento de recomendaciones es proporcionar al clínico la mejor evidencia disponible y, en su defecto, la mejor opinión consensuada por los panelistas para un uso racional y fundado de las diversas opciones de tratamiento con fármacos antirreumáticos modificadores de la enfermedad (FAME) sintéticos y biológicos en artropatía psoriásica (APs). El presente documento también incide sobre aspectos importantes en el manejo de la APs, como el diagnóstico precoz, los objetivos terapéuticos, las comorbilidades y la optimización del tratamiento. Métodos: Las recomendaciones se consensuaron a través de un panel de 8 reumatólogos expertos, previamente seleccionados por la Sociedad Española de Reumatología (SER) mediante una convocatoria abierta. Las fases del trabajo fueron: identificación de las áreas claves 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: Se emiten un total de 17 recomendaciones para el tratamiento de los pacientes con APs. Seis de ellas de carácter general, que abarcan desde la transcendencia del diagnóstico y tratamiento precoz hasta la importancia de las comorbilidades. El resto, las 11 específicas, se centran en las indicaciones de los FAME y la terapia biológica en las diferentes formas clínicas de la enfermedad. Así mismo, se abordan las situaciones de fracaso a un primer biológico y se incluyen los algoritmos de tratamientos y una tabla con las diferentes terapias biológicas. Conclusiones: Se presenta la actualización de las recomendaciones de la SER para el tratamiento de la APs con FAME y terapia biológica
Objective: The main purpose of this recommendation statement is to provide clinicians with the best available evidence and the best opinion agreed upon by the panelists for a rational use of synthetic disease modifying antirheumatic drugs (DMARDs) and biologicals in psoriatic arthritis (PsA) patients. The present document also focuses on important aspects in the management of PsA, such as early diagnosis, therapeutic objectives, comorbidities and optimization of treatment. Methods: The recommendations were agreed by consensus by a panel of 8 expert rheumatologists, previously selected by the Spanish Society of Rheumatology (SER) through an open call. The phases of the work were: identification of key areas for updating the previous consensus, analysis and synthesis of scientific evidence (modified Oxford system, Centre for Evidence-based Medicine, 2009) and formulation of recommendations based on this evidence and by consensus techniques. Results: Seventeen recommendations were issued for the treatment of PsA patients. Six of them were of general nature, ranging from the early diagnosis and treatment to the importance of assessing comorbidities. The other 11 were focused on the indications for DMARDs and biological therapy in the distinct clinical forms of the disease. Likewise, the situation of failure of the first biological is addressed and treatment algorithms and a table with the different biological therapies are also included. Conclusions: We present the update of SER recommendations for the treatment of PsA with DMARDs and biologics
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Humanos , Artrite Psoriásica/tratamento farmacológico , Terapia Biológica , Antirreumáticos/uso terapêutico , Padrões de Prática MédicaRESUMO
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.
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Espondilite Anquilosante/diagnóstico , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores Sexuais , Fatores de TempoRESUMO
Registries estimate that one third of patients with psoriatic arthritis (PsA) are "resistant" to of TNF-alpha blockers. Therefore, the search for new approaches to treatment of this disease may be justified. Currently the treatment options that have proven effective are associated with inhibition of the T cell costimulatory pathway (abatacept and alefacept) and blocking the P40 fraction of IL-12 and IL-23 (ustekinumab). A novel pathway inhibition, which deserves special attention is offered by apremilast. This molecule inhibits phosphodiesterase IV, responsible for hydrolyzing cyclic adenosine monophosphate to adenosine monophosphate, which causes an increase in cAMP. This metabolite is associated with decreased TNF-alpha. It has a modest efficacy (ACR 20 response of 43%), and subsequent studies have shown an improvement in visual analog scale and the SF36 compared to placebo. Currently there are five clinical trials in phase III to assess its effectiveness in parameters of inflammation and radiographic progression. The spectrum of possibilities before treatment failure with anti-TNF alpha, is augmented by the appearance of several reports that show efficacy with the individual use of CD20 inhibitors and IL-1. In patients with rheumatoid arthritis (RA) the effectiveness of molecules that inhibit signal transduction of cytokines (Anti-JAK) has been proven, so it is possible that in the future they may be used in patients with PsA.
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Artrite Psoriásica/tratamento farmacológico , Terapia de Alvo Molecular , Aminopiridinas , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados , Antígenos CD20/efeitos dos fármacos , Antirreumáticos/uso terapêutico , Artrite Psoriásica/metabolismo , Artrite Psoriásica/patologia , Remodelação Óssea/efeitos dos fármacos , Ensaios Clínicos Fase III como Assunto , AMP Cíclico/fisiologia , Citocinas/antagonistas & inibidores , Denosumab , Progressão da Doença , Resistência a Medicamentos , Humanos , Inflamação , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Interleucina-1 , Janus Quinases/antagonistas & inibidores , Ativação Linfocitária/efeitos dos fármacos , Depleção Linfocítica , Morfolinas , Oxazinas/uso terapêutico , Inibidores da Fosfodiesterase 4 , Piridinas/uso terapêutico , Pirimidinas , Subpopulações de Linfócitos T/efeitos dos fármacos , Subpopulações de Linfócitos T/imunologia , Talidomida/análogos & derivados , Talidomida/uso terapêutico , Resultado do Tratamento , Fator de Necrose Tumoral alfa/antagonistas & inibidoresRESUMO
No disponible
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Humanos , Masculino , Pessoa de Meia-Idade , Ocronose/complicações , Ocronose/diagnóstico , Ocronose/terapia , Osteoporose/complicações , Osteoporose/terapia , Osteoporose , Espondilartrite/complicações , Espondilartrite/terapia , Difosfonatos/uso terapêutico , Alendronato/uso terapêutico , Alcaptonúria/complicações , Espondilartrite , Insuficiência Respiratória/complicações , Insuficiência Renal/complicaçõesRESUMO
El síndrome SAPHO (sinovitis, acné, pustulosis, hiperostosis y osteítis) comprende un conjunto de manifestaciones cutáneo-osteoarticulares. Se han descrito algunas complicaciones graves que pueden aparecer durante la evolución de la enfermedad, como la trombosis venosa, principalmente en pacientes que desarrollan afectación inflamatoria grave de la pared torácica anterior. El objetivo de la presente revisión fue analizar los casos descritos en la literatura médica relacionados con la presencia de complicaciones trombóticas en pacientes diagnosticados de síndrome SAPHO e intentar establecer los probables factores de riesgo y su posible mecanismo patogénico. Se analizaron 11 artículos publicados de casos clínicos aislados o series de casos, con un total de 144 pacientes, que describen en total 15 casos de trombosis venosa. Se exponen las características clínicas de estos pacientes, se evalúa si cumplen los criterios de clasificación ASAS para espondiloatritis axial y periférica, y se resalta la necesidad de realizar un diagnóstico y tratamiento precoces (AU)
SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted (AU)
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/epidemiologia , Fatores de Risco , Trombose Venosa/complicações , Trombose Venosa/terapia , Trombose Venosa , Diagnóstico Precoce , Anticoagulantes/uso terapêutico , Síndrome de Hiperostose Adquirida/fisiopatologia , Síndrome de Hiperostose Adquirida , Veia Subclávia/patologia , Veia Subclávia , Tomografia Computadorizada de Emissão , Cintilografia/métodosRESUMO
Los registros estiman que un tercio de los pacientes con artritis psoriásica (Aps) son «resistentes» a los bloqueantes del TNF-alfa. Por ello, la búsqueda de nuevos abordajes terapéuticos de la enfermedad es un objetivo que se puede considerar justificado. Actualmente las opciones terapéuticas que han probado su eficacia, son las vinculadas a la inhibición de la vía coestimuladora del linfocito T (abatacept y alefacept) y el bloqueo de la fracción P40 de la IL-12 e IL-23 (ustekinumab). Una novedosa vía de inhibición, que merece especial atención, es la que ofrece Apremilast. Esta molécula inhibe la fosfodiesterasa IV encargada de hidrolizar la adenosina monofosfato cíclica a adenosina monofosfato, lo que provoca un aumento de la cAMP. Este metabolito se relaciona con una disminución del TNF alfa. Capaz de provocar una modesta eficacia (respuesta ACR 20 del 43%), estudios posteriores han demostrado una mejoría en la escala visual analógica y en el SF36 respecto al grupo placebo. Actualmente hay en marcha 5 ensayos clínicos en fase III que evaluarán su eficacia en parámetros de inflamación y de progresión radiográfica. El espectro de posibilidades, ante el fracaso terapéutico con anti-TNF alfa, se amplía con la aparición de diversos reportes donde se ha mostrado eficacia en la utilización individual con agentes inhibidores del CD20 y de la IL-1. Se está demostrando en pacientes con artritis reumatoide (AR) la eficacia de las moléculas que inhiben la transducción de las señales de las citocinas (Anti-JAK), por lo que es posible que en un futuro sean utilizadas en pacientes con Aps (AU)
Registries estimate that one third of patients with psoriatic arthritis (PsA) are "resistant" to of TNF-alpha blockers. Therefore, the search for new approaches to treatment of this disease may be justified. Currently the treatment options that have proven effective are associated with inhibition of the T cell costimulatory pathway (abatacept and alefacept) and blocking the P40 fraction of IL-12 and IL-23 (ustekinumab). A novel pathway inhibition, which deserves special attention is offered by apremilast. This molecule inhibits phosphodiesterase IV, responsible for hydrolyzing cyclic adenosine monophosphate to adenosine monophosphate, which causes an increase in cAMP. This metabolite is associated with decreased TNF-alpha. It has a modest efficacy (ACR 20 response of 43%), and subsequent studies have shown an improvement in visual analog scale and the SF36 compared to placebo. Currently there are five clinical trials in phase III to assess its effectiveness in parameters of inflammation and radiographic progression. The spectrum of possibilities before treatment failure with anti-TNF alpha, is augmented by the appearance of several reports that show efficacy with the individual use of CD20 inhibitors and IL-1. In patients with rheumatoid arthritis (RA) the effectiveness of molecules that inhibit signal transduction of cytokines (Anti-JAK) has been proven, so it is possible that in the future they may be used in patients with PsA (AU)