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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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INTRODUCTION: Obstetric complications are more common in women with systemic lupus erythematosus (SLE) than in the general population. OBJECTIVE: To assess pregnancy outcomes in women with SLE from the RELESSER cohort after 12 years of follow-up. METHODS: A multicentre retrospective observational study was conducted. In addition to data from the RELESSER register, data were collected on obstetric/gynaecological variables and treatments received. The number of term pregnancies was compared between women with pregnancies before and after the diagnosis of SLE. Further, clinical and laboratory characteristics were compared between women with pregnancies before and after the diagnosis, on the one hand, and with and without complications during pregnancy, on the other. Bivariate and multivariate analyses were carried out to identify factors potentially associated with complications during pregnancy. RESULTS: A total of 809 women were included, with 1869 pregnancies, of which 1395 reached term. Women with pregnancies before the diagnosis of SLE had more pregnancies (2.37 vs 1.87) and a higher rate of term pregnancies (76.8% vs 69.8%, p < 0.001) compared to those with pregnancies after the diagnosis. Women with pregnancies before the diagnosis were diagnosed at an older age (43.4 vs 34.1 years) and had more comorbidities. No differences were observed between the groups with pregnancies before and after diagnosis in antibody profile, including anti-dsDNA, anti-Sm, anti-Ro, anti-La, lupus anticoagulant, anticardiolipin or anti-beta-2-glycoprotein. Overall, 114 out of the 809 women included in the study experienced complications during pregnancy, including miscarriage, preeclampsia/eclampsia, foetal death, and/or preterm birth. Women with complications had higher rates of antiphospholipid syndrome (40.5% vs 9.9%, p < 0.001) and higher rates of positivity for IgG anticardiolipin (33.9% vs 21.3%, p = 0.005), IgG anti-beta 2 glycoprotein (26.1% vs 14%, p = 0.007), and IgM anti-beta 2 glycoprotein (26.1% vs 16%, p = 0.032) antibodies, although no differences were found regarding lupus anticoagulant. Among the treatments received, only heparin was more commonly used by women with pregnancy complications. We did not find differences in corticosteroid or hydroxychloroquine use. CONCLUSIONS: The likelihood of term pregnancy is higher before the diagnosis of SLE. In our cohort, positivity for anticardiolipin IgG and anti-beta-2- glycoprotein IgG/IgM, but not lupus anticoagulant, was associated with a higher risk of poorer pregnancy outcomes.
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Síndrome Antifosfolípido , Lupus Eritematoso Sistémico , Complicaciones del Embarazo , Nacimiento Prematuro , Reumatología , Embarazo , Humanos , Recién Nacido , Femenino , Resultado del Embarazo/epidemiología , Nacimiento Prematuro/epidemiología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Síndrome Antifosfolípido/complicaciones , Complicaciones del Embarazo/epidemiología , Estudios Retrospectivos , beta 2 Glicoproteína I , Anticoagulantes , Inmunoglobulina G , Inmunoglobulina MRESUMEN
Systemic sclerosis (SSc) is a chronic autoimmune disease with complex pathogenesis, characterized by vascular dysfunction and fibrosis. Digital ulcers (DUs) are a common and severe complication in SSc patients, negatively impacting their quality of life. This retrospective study evaluates the use of macitentan, an endothelin receptor antagonist, in six female patients with connective tissue disease (CTD) and sclerodermiform features (five SSc and one mixed connective tissue disease) for the treatment of refractory DUs. Macitentan demonstrated a safe and effective alternative to bosentan, reducing DU relapses, hospitalizations, and the use of systemic prostaglandin therapy. The findings suggest that macitentan may be a valuable therapeutic option in specific cases of recurrent or refractory DUs and warrant further investigation in larger, long-term studies.
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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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Lupus Eritematoso Sistémico , Vasculitis por Lupus del Sistema Nervioso Central , Reumatología , Humanos , Estudios Retrospectivos , Lupus Eritematoso Sistémico/epidemiología , Vasculitis por Lupus del Sistema Nervioso Central/complicaciones , Vasculitis por Lupus del Sistema Nervioso Central/epidemiología , Vasculitis por Lupus del Sistema Nervioso Central/psicología , Sistema Nervioso CentralRESUMEN
OBJETIVOS: Analizar las características clínicas y de metabolismo óseo de una serie de pacientes con fracturas vertebrales tras la suspensión de denosumab (DMab). MÉTODOS: Estudio observacional retrospectivo de 10 pacientes con fracturas vertebrales tras suspender DMab atendidas en el Servicio de Reumatología de un hospital español de tercer nivel entre 2015 y 2018. RESULTADOS: Se registraron un total de 49 fracturas espontáneas tras una media de 6 dosis de DMab y transcurridos 10,9 meses desde la suspensión del fármaco. El 90% había recibido tratamiento previo, 7 de 10 bisfosfonatos orales. Tras la suspensión, CTX y P1NP estaban elevados y la media de T-score en cuello femoral y columna lumbar fue menor que previo a DMab. Las vértebras más afectadas fueron L3, L5, D6, D7, D9 y D11. CONCLUSIÓN: La descripción de nuevos casos de fracturas vertebrales múltiples en los meses posteriores a la suspensión de DMab subraya la preocupación emergente en la comunidad científica siendo preciso apoyar en evidencias sólidas las nuevas recomendaciones sobre su manejo
OBJECTIVES: Analyse clinical and bone metabolism features in a case series of patients with multiple vertebral fractures after discontinuation of denosumab (DMab). METHODS: An observational descriptive study analysing data from ten patients with multiple vertebral fractures after DMab discontinuation that were admitted to our rheumatology department between 2015 and 2018. RESULTS: There were a total of 49 spontaneous fractures after an average of 6 DMab doses and 10.9 months from discontinuation. Ninety percent had already received treatment other than DMab 7 of 10 oral bisphosphonates. After discontinuation, CTX and P1NP remained elevated and mean T-score for femoral neck and lumbar spine was lower than before treatment. The most affected vertebrae were L3, L5, D6, D7, D9 and D11. CONCLUSION: This report of ten new cases suffering multiple vertebral fractures early after discontinuation of DMab highlights the emerging concern on the subject in the scientific community and the need to clarify its pathogenic mechanism, and to support by solid evidence the new recommendations on its management
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Humanos , Femenino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Denosumab/uso terapéutico , Fracturas Múltiples/etiología , Fracturas Osteoporóticas/epidemiología , Fracturas de la Columna Vertebral/epidemiología , Conservadores de la Densidad Ósea/uso terapéutico , Privación de Tratamiento , Estudios Retrospectivos , Efecto Rebote , Anticuerpos Monoclonales Humanizados/uso terapéutico , Densitometría/métodosRESUMEN
OBJECTIVES: Analyse clinical and bone metabolism features in a case series of patients with multiple vertebral fractures after discontinuation of denosumab (DMab). METHODS: An observational descriptive study analysing data from ten patients with multiple vertebral fractures after DMab discontinuation that were admitted to our rheumatology department between 2015 and 2018. RESULTS: There were a total of 49 spontaneous fractures after an average of 6 DMab doses and 10.9 months from discontinuation. Ninety percent had already received treatment other than DMab 7 of 10 oral bisphosphonates. After discontinuation, CTX and P1NP remained elevated and mean T-score for femoral neck and lumbar spine was lower than before treatment. The most affected vertebrae were L3, L5, D6, D7, D9 and D11. CONCLUSION: This report of ten new cases suffering multiple vertebral fractures early after discontinuation of DMab highlights the emerging concern on the subject in the scientific community and the need to clarify its pathogenic mechanism, and to support by solid evidence the new recommendations on its management.
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Conservadores de la Densidad Ósea/administración & dosificación , Denosumab/administración & dosificación , Fracturas Múltiples/etiología , Fracturas de la Columna Vertebral/etiología , Privación de Tratamiento , Anciano , Femenino , Humanos , Persona de Mediana EdadRESUMEN
OBJECTIVES: To assess the plasma apolipoprotein B/apolipoprotein A1 ratio and its potential association with cardiovascular events (CVE) in patients with rheumatoid arthritis (RA). METHODS: A baseline analysis was made of the CARdiovascular in rheuMAtology Project (CARMA), a 10-year prospective study evaluating the presence of at least one CVE in 775 Spanish patients with RA. Of them, 29 had already experienced CVE prior to the inclusion in the study. We assessed the association between the elevation of the apoB/apoA1 ratio with the presence of CVE according to a logistic regression model for possible confounding factors. We also analysed the main parameters of activity of RA and parameters related to lipid metabolism. RA patients were classified according to treatment: patients treated with disease-modifying anti-rheumatic drugs without biologics and those undergoing biologic therapy (anti-TNF-α, anti-IL-6 receptor, and other biologic agents). RESULTS: The apoB/apoA1 ratio of patients who had experienced CVE was higher than that of patients without previous CVE (0.65 vs. 0.60). However, the difference between both subgroups did not reach statistical significance (p=0.197). It was also the case after the multivariate analysis [OR: 1.48 (95% CI: 0.15-14.4); p=0.735]. RA patients from the group with CVE were more commonly receiving lipid-lowering treatment with statins than those without CVE history (41.4% vs. 20%, p=0.005). High HAQ and high atherogenic index were significantly associated with the presence of CVE. There was no statistical association between the type of biologic therapy used in RA and the presence of CVE. CONCLUSIONS: No association between ApoB/apoA1 ratio and CVE was found at the baseline visit of patients with RA from the CARMA study.
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Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Enfermedades Cardiovasculares , Apolipoproteína A-I , Apolipoproteínas B , Humanos , Estudios Prospectivos , Factor de Necrosis Tumoral alfa/uso terapéuticoRESUMEN
La enfermedad gonocócica diseminada es una manifestación infrecuente de la afectación por Neisseria gonorrhoeae, que presenta una clínica variada y no bien definida, siendo la afectación articular un hallazgo característico. Presentamos el caso de una mujer de 64 años con enfermedad gonocócica diseminada de inicio agudo, que comenzó con deterioro generalizado y oligoartritis. Se realizó artrocentesis de carpo, obteniéndose un líquido sinovial de aspecto purulento, cuyo estudio microbiológico identificó Neisseira gonorrhoeae. En el estudio se objetivó un complemento hemolítico total (CH50) de cero, no detectándose la fracción C2 del complemento. Son muy pocos los casos descritos en la literatura de enfermedad gonocócica diseminada asociada a déficit de C2. Presentamos un nuevo caso y revisamos los previamente publicados
Disseminated gonococcal infection is a rare presentation of the sexually transmitted pathogen, Neisseria gonorrhoeae. Here, we report the case of a 64-year-old woman with disseminated gonococcal infection, which started with symptoms of oligoarthritis and malaise. Neisseria gonorrhoeae was identified in the carpal synovial fluid. The follow-up study revealed an absence of total hemolytic complement and complement C2 was not detected. Being relatively common, C2 deficiency has been associated with disseminated gonococcal infection in a few cases. We present a new case and discuss those previously published
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Humanos , Femenino , Persona de Mediana Edad , Artritis Infecciosa/microbiología , Complemento C2/deficiencia , Gonorrea/complicacionesRESUMEN
Disseminated gonococcal infection is a rare presentation of the sexually transmitted pathogen, Neisseria gonorrhoeae. Here, we report the case of a 64-year-old woman with disseminated gonococcal infection, which started with symptoms of oligoarthritis and malaise. Neisseria gonorrhoeae was identified in the carpal synovial fluid. The follow-up study revealed an absence of total hemolytic complement and complement C2 was not detected. Being relatively common, C2 deficiency has been associated with disseminated gonococcal infection in a few cases. We present a new case and discuss those previously published.
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Artritis Infecciosa/microbiología , Complemento C2/deficiencia , Gonorrea/complicaciones , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Las enfermedades del colágeno constituyen un gran grupo de enfermedades inflamatorias sistémicas de etiología autoinmune. La etiopatogenia de las enfermedades del colágeno es multifactorial. Existe una susceptibilidad genética, y muchos trastornos del tejido conectivo muestran una agregación familiar, sobre la que actúan factores ambientales que desencadenan la enfermedad. En las enfermedades del colágeno se pueden afectar casi todos los órganos del cuerpo. El sistema respiratorio es uno de los más frecuentemente afectados, aunque no se conoce con exactitud la prevalencia de enfermedad pulmonar en las diferentes enfermedades del colágeno. Cualquier estructura del aparato respiratorio puede estar afectada. Quizá lo más frecuente sea la enfermedad del parénquima pulmonar en forma de neumonitis, que puede manifestarse como cualquiera de los patrones de neumonitis intersticiales idiopáticas. Pero también pueden afectarse la pleura, los vasos pulmonares, la vía aérea y la musculatura respiratoria. La frecuencia de enfermedad pulmonar asociada a las enfermedades del colágeno está aumentando, por una parte gracias a los mejores métodos de diagnóstico de que disponemos hoy en día, como la tomografía computarizada de alta resolución, y también por la aparición de nuevas formas de neumonitis asociadas a los nuevos tratamientos empleados en la actualidad. El objetivo de este artículo es ofrecer una visión en conjunto de cómo las enfermedades del colágeno pueden afectar el pulmón, de acuerdo con las nuevas evidencias científicas (AU)
Collagen diseases are a large group of systemic inflammatory diseases of autoimmune etiology. The etiopathogenesis of collagen diseases is multifactorial. There is genetic susceptibility, as many connective tissue disorders show family history, and environmental factors may trigger the disease. Collagen diseases can affect almost all the organs of the body. The respiratory system is one of the most frequently affected, although the prevalence of pulmonary disease is not precisely known for the different collagen disorders. Any structure of the respiratory tract can be affected, but perhaps the most frequent is pulmonary parenchymal disease in the form of pneumonitis, which can be produced in any of the idiopathic interstitial pneumonitis patterns. The pleura, pulmonary vessels, airways and respiratory muscles may also be affected. The frequency of lung disease associated with collagen diseases is on the rise. This is in due part to the better diagnostic methods that are available to us today (such as high-resolution computed tomography) and also to the appearance of new forms of pneumonitis associated with the new treatments that are currently used. The objective of this article is to offer a global vision of how collagen diseases can affect the lungs according to the latest scientific evidence (AU)
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Humanos , Enfermedades del Colágeno/complicaciones , Enfermedades Pulmonares Intersticiales/complicaciones , Artritis Reumatoide/complicaciones , Esclerodermia Sistémica/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Miositis/complicaciones , Enfermedades del Tejido Conjuntivo/complicacionesRESUMEN
Collagen diseases are a large group of systemic inflammatory diseases of autoimmune etiology. The etiopathogenesis of collagen diseases is multifactorial. There is genetic susceptibility, as many connective tissue disorders show family history, and environmental factors may trigger the disease. Collagen diseases can affect almost all the organs of the body. The respiratory system is one of the most frequently affected, although the prevalence of pulmonary disease is not precisely known for the different collagen disorders. Any structure of the respiratory tract can be affected, but perhaps the most frequent is pulmonary parenchymal disease in the form of pneumonitis, which can be produced in any of the idiopathic interstitial pneumonitis patterns. The pleura, pulmonary vessels, airways and respiratory muscles may also be affected. The frequency of lung disease associated with collagen diseases is on the rise. This due in part to the better diagnostic methods that are available to us today (such as high-resolution computed tomography) and also to the appearance of new forms of pneumonitis associated with the new treatments that are currently used. The objective of this article is to offer a global vision of how collagen diseases can affect the lungs according to the latest scientific evidence.
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Enfermedades del Colágeno/complicaciones , Enfermedades Pulmonares/etiología , Bronquiectasia/etiología , Bronquiolitis Obliterante/etiología , Síndrome de Caplan/etiología , Enfermedades del Colágeno/fisiopatología , Humanos , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares/fisiopatología , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Trastornos Linfoproliferativos/etiología , Derrame Pleural/etiología , Neumonía/etiología , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/fisiopatologíaRESUMEN
OBJECTIVE: To investigate the frequency and pattern of presentation of uveitis as the first clinical manifestation to prompt diagnostic evaluation in patients with spondyloarthropathies (SpA). METHODS: Patients with uveitis were attended simultaneously by ophthalmologists and rheumatologists in our Uveitis Clinic between June 1997 and October 2000. An established clinical protocol based on the pattern of uveitis and the patient's symptoms was used to determine diagnosis. Evaluation included clinical history, ophthalmologic examination, hemogram, biochemistry, erythrocyte sedimentation rate, the fluorescent treponemal antibody absorption test, urinalysis, and chest radiograph. Additional studies were requested according to the protocol. RESULTS: Data from 394 patients were recorded in our database. Seventy-two (18%) had some type of SpA; their mean age was 44.7 years (SD 15.7) and 51 (71.8%) were men. Forty-two patients (59%) of the SpA group had been previously diagnosed. In the 30 (41%) who were undiagnosed, uveitis was the first manifestation to prompt diagnostic evaluation. The most frequent clinical pattern was acute unilateral anterior uveitis. The 2 main keys to confirm the diagnosis of SpA were the presence of recurrent acute unilateral uveitis and low back or joint pain, in addition to the uveitis flare. HLA-B27 was found in 94% of patients. CONCLUSION: In 41% of the patients diagnosed with SpA, uveitis was the first clinical sign, suggesting that collaboration between ophthalmologists and rheumatologists greatly aids the diagnosis and treatment of these patients. When this close collaboration is not possible, all patients with rheumatic complaints and recurrent acute unilateral uveitis should be referred to a rheumatologist.