Prevalence and clinical impact of systemic autoimmune rheumatic disease in patients with silicosis / Prevalencia e impacto clínico de las enfermedades reumatológicas autoinmunitarias sistémicas en pacientes con silicosis

Background: Silicosis is associated with an increased risk of developing systemic autoimmune rheumatic disease (SARD). The prognostic implications of this association are poorly characterized. The aim of this study was to determine the prevalence of SARD and autoimmune markers in a cohort of patients with exposure to silica and assess their impact on prognosis. Method: We performed a prospective observational study of all patients attending the dedicated silicosis clinic of our pulmonology unit between 2009 and December 2017. Diagnosis was confirmed by a rheumatologist according to Spanish Rheumatology Society criteria. Autoimmune markers, pulmonary function tests, radiological progression, visits to the emergency department and primary care center, and hospital admissions for respiratory causes, and mortality were analyzed. Results: Overall, 489 cases of silicosis and 95 cases of exposure were studied. In total, 54 (11.0%) patients with silicosis had SARD: 12 (2.4%) rheumatoid arthritis, 10 (2.0%) systemic lupus erythematosus, 10 (2.0%) systemic sclerosis, 3 (0.6%) Sjögren syndrome, 2 (0.4%) vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA +), 6 (1.2%) psoriatic arthritis, 3 (0.6%) ankylosing spondylitis, and 8 (1.6%) other autoimmune diseases with no special features. The patients with SARD visited the emergency room more often (63.0% vs. 42.5%; p = 0.004), and progressed more rapidly (22.2 vs. 11.7%; p = 0.030). Conclusions: The presence of systemic rheumatic autoimmune diseases involves radiological progression and a higher clinical impact. (AU)

Introducción: La silicosis se asocia a un aumento del riesgo de padecer una de las enfermedades reumatológicas autoinmunes sistémicas (ERAS), aunque no se conocen las implicaciones clínicas de esta asociación. El objetivo del estudio es determinar la prevalencia de ERAS y de marcadores de autoinmunidad en una cohorte de pacientes con exposición a inhalación de polvo de sílice y evaluar su impacto clínico. Método: Estudio observacional prospectivo en pacientes atendidos en una consulta monográfica de silicosis desde 2009 hasta diciembre 2017. El diagnóstico de ERAS se confirmó por un especialista en Reumatología según criterios de la Sociedad Española de Reumatología. Se analizaron marcadores de autoinmunidad, pruebas de función respiaratoria, progresión radiológica e impacto clínico medido por visitas a Atención Primaria, a Servicio de Urgencias, ingresos hospitalarios por causa respiratoria y mortalidad. Resultados: Se estudiaron 489 casos de silicosis y 95 de exposición a inhalación de polvo de sílice sin silicosis. De los pacientes con silicosis, 54 (11,0%) tenían ERAS: 12 (2,4%) artritis reumatoide, 10 (2,0%) lupus eritematoso sistémico, 10 (2,0%) esclerosis sistémica, 6 (1,2%) artritis psoriásica, 3 (0,6%) Síndrome de Sjögren, 2 (0,4%) vasculitis asociada a anticuerpos anticitoplasma de neutrófilos, 3 (0,6%) espondiloartritis y 8 (1,6%) enfermedad autoinmune sin características específicas. Los pacientes con ERAS realizaron más visitas a urgencias (63,0% vs. 42,5%; p = 0,004), y experimentaron mayor progresión (22,2 vs. 11,7%; p = 0,030). Conclusiones: Los pacientes con silicosis presentan una prevalencia de ERAS elevada y su presencia se asocia a una mayor progresión radiológica y un mayor impacto clínico. (AU)

Prevalence and clinical impact of systemic autoimmune rheumatic disease in patients with silicosis.

BACKGROUND: Silicosis is associated with an increased risk of developing systemic autoimmune rheumatic disease (SARD). The prognostic implications of this association are poorly characterized. The aim of this study was to determine the prevalence of SARD and autoimmune markers in a cohort of patients with exposure to silica and assess their impact on prognosis. METHOD: We performed a prospective observational study of all patients attending the dedicated silicosis clinic of our pulmonology unit between 2009 and December 2017. Diagnosis was confirmed by a rheumatologist according to Spanish Rheumatology Society criteria. Autoimmune markers, pulmonary function tests, radiological progression, visits to the emergency department and primary care center, and hospital admissions for respiratory causes, and mortality were analyzed. RESULTS: Overall, 489 cases of silicosis and 95 cases of exposure were studied. In total, 54 (11.0%) patients with silicosis had SARD: 12 (2.4%) rheumatoid arthritis, 10 (2.0%) systemic lupus erythematosus, 10 (2.0%) systemic sclerosis, 3 (0.6%) Sjögren syndrome, 2 (0.4%) vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA +), 6 (1.2%) psoriatic arthritis, 3 (0.6%) ankylosing spondylitis, and 8 (1.6%) other autoimmune diseases with no special features. The patients with SARD visited the emergency room more often (63.0% vs. 42.5%; p = 0.004), and progressed more rapidly (22.2 vs. 11.7%; p = 0.030). CONCLUSIONS: The presence of systemic rheumatic autoimmune diseases involves radiological progression and a higher clinical impact.

Prevalence and Clinical Impact of Systemic Autoimmune Rheumatic Disease in Patients with Silicosis. / Prevalencia e impacto clínico de las enfermedades reumatológicas autoinmunitarias sistémicas en pacientes con silicosis.

BACKGROUND: Silicosis is associated with an increased risk of developing systemic autoimmune rheumatic disease (SARD). The prognostic implications of this association are poorly characterized. The aim of this study was to determine the prevalence of SARD and autoimmune markers in a cohort of patients with exposure to silica and assess their impact on prognosis. METHOD: We performed a prospective observational study of all patients attending the dedicated silicosis clinic of our pulmonology unit between 2009 and December 2017. Diagnosis was confirmed by a rheumatologist according to Spanish Rheumatology Society criteria. Autoimmune markers, pulmonary function tests, radiological progression, visits to the emergency department and primary care center, and hospital admissions for respiratory causes, and mortality were analyzed. RESULTS: Overall, 489 cases of silicosis and 95 cases of exposure were studied. In total, 54 (11.0%) patients with silicosis had SARD: 12 (2.4%) rheumatoid arthritis, 10 (2.0%) systemic lupus erythematosus, 10 (2.0%) systemic sclerosis, 3 (0.6%) Sjögren syndrome, 2 (0.4%) vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA +), 6 (1.2%) psoriatic arthritis, 3 (0.6%) ankylosing spondylitis, and 8 (1.6%) other autoimmune diseases with no special features. The patients with SARD visited the emergency room more often (63.0 vs. 42.5%; p = 0.004), and progressed more rapidly (22.2 vs. 11.7%; p = 0.030). CONCLUSIONS: The presence of systemic rheumatic autoimmune diseases involves radiological progression and a higher clinical impact.

La prueba de la marcha de 6 minutos como factor pronóstico y de capacidad de ejercicio en pacientes con silicosis / The 6-Minute Walk Test as a Tool for Determining Exercise Capacity and Prognosis in Patients with Silicosis

Objetivo: Evaluar la capacidad de ejercicio, utilizando la prueba de la marcha de 6 minutos (6MWT), en pacientes con silicosis complicada, e identificar factores relacionados con dicho resultado, así como su valor pronóstico de hospitalización y mortalidad. Método: Estudio observacional prospectivo en 143 pacientes diagnosticados de silicosis complicada atendidos en consulta de neumología desde 2009 hasta diciembre 2016, sin limitación para hacer la 6MWT. Se evaluaron las pruebas de función pulmonar, los hallazgos de la tomografía computarizada y los parámetros de la 6MWT (distancia caminada y desaturación de oxígeno). Resultados: Los pacientes con silicosis complicada caminaban una media de 387 ± 92m. Los resultados fueron peores al aumentar la gravedad de la enfermedad; en silicosis A: 407 ± 97; B: 370 ± 88, y C: 357 ± 68 (p=0,022). La 6MWT mostraba una correlación positiva con la función pulmonar e inversa con la disnea (p < 0,001). Los pacientes con enfisema moderado/severo y un mayor tiempo con saturación nocturna de oxígeno por debajo del 90% presentaban resultados significativamente peores en la 6MWT. Los pacientes que caminaban < 350 m tenían más hospitalizaciones (3,7 ± 4,0) que los que caminaban>350 (1,1 ± 2,9). Los fallecidos al final del seguimiento caminaron 327,9 ± 73,0 frente a los que sobreviven, que caminaron 404,5 ± 89,7 m (p < 0,001). Conclusiones: Los valores de la espirometría y la categoría de la silicosis se relacionan con el resultado de la 6MWT, que resultó ser factor pronóstico para hospitalización y mortalidad. La 6MWT se perfila como una herramienta útil en salud ocupacional para el seguimiento de pacientes con silicosis

Objective: To evaluate exercise capacity using the 6-minute walk test (6MWT) in patients with complicated silicosis, and to determine how results shape the prognostic value of 6MWT for hospitalization and mortality. Method: Prospective observational study in 143 patients with complicated silicosis who performed the 6MWT in our outpatient pulmonology clinic between 2009 and the end of 2016. Lung function tests, computed tomography findings and 6MWT parameters (distance walked and oxygen desaturation) were evaluated. Results: Patients with complicated silicosis walked 387±92m. Results deteriorated as silicosis severity increased; category A: 407 ± 97m, B: 370±88m, and C: 357 ± 68m (P=.022). The 6MWT correlated positively with lung function and inversely with dyspnea determined by mMRC (P < .001). Patients with moderate/severe emphysema and greater CT90 presented a worse result on 6MWT. Patients who walked < 350m had more hospitalizations (3.7 ± 4.0) than those who walked > 350m (1.1 ± 2.9). There was an impact on survival: non-survivors walked 327.9 ± 73.0m, while survivors walked 404.5 ± 89.7m (P < .001). Conclusions: Spirometry values and category of complicated silicosis correlated with 6MWT and distance walked was a prognostic factor for hospitalization and mortality. The 6MWT is a useful tool in occupational health for monitoring patients with silicosis

The 6-Minute Walk Test as a Tool for Determining Exercise Capacity and Prognosis in Patients with Silicosis. / La prueba de la marcha de 6 minutos como factor pronóstico y de capacidad de ejercicio en pacientes con silicosis.

OBJECTIVE: To evaluate exercise capacity using the 6-minute walk test (6MWT) in patients with complicated silicosis, and to determine how results shape the prognostic value of 6MWT for hospitalization and mortality. METHOD: Prospective observational study in 143 patients with complicated silicosis who performed the 6MWT in our outpatient pulmonology clinic between 2009 and the end of 2016. Lung function tests, computed tomography findings and 6MWT parameters (distance walked and oxygen desaturation) were evaluated. RESULTS: Patients with complicated silicosis walked 387±92m. Results deteriorated as silicosis severity increased; category A: 407±97m, B: 370±88m, and C: 357±68m (P=.022). The 6MWT correlated positively with lung function and inversely with dyspnea determined by mMRC (P<.001). Patients with moderate/severe emphysema and greater CT90 presented a worse result on 6MWT. Patients who walked<350m had more hospitalizations (3.7±4.0) than those who walked>350m (1.1±2.9). There was an impact on survival: non-survivors walked 327.9±73.0m, while survivors walked 404.5±89.7m (P<.001). CONCLUSIONS: Spirometry values and category of complicated silicosis correlated with 6MWT and distance walked was a prognostic factor for hospitalization and mortality. The 6MWT is a useful tool in occupational health for monitoring patients with silicosis.

Pulmonary vein stenosis: Etiology, diagnosis and management.

Pulmonary vein stenosis (PVS) is rare condition characterized by a challenging diagnosis and unfavorable prognosis at advance stages. At present, injury from radiofrequency ablation for atrial fibrillation has become the main cause of the disease. PVS is characterized by a progressive lumen size reduction of one or more pulmonary veins that, when hemodynamically significant, may raise lobar capillary pressure leading to signs and symptoms such as shortness of breath, cough, and hemoptysis. Image techniques (transesophageal echocardiography, computed tomography, magnetic resonance and perfusion imaging) are essential to reach a final diagnosis and decide an appropriate therapy. In this regard, series from referral centers have shown that surgical and transcatheter interventions may improve prognosis. The purpose of this article is to review the etiology, assessment and management of PVS.