Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes / Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients

Introducción: El síndrome de pulmón encogido (SPE) es una manifestación rara del lupus eritematoso sistémico. Nuestro objetivo fue describir las características clínicas, radiológicas y funcionales de una cohorte con SPE y su evolución en el tiempo.MétodosEstudio retrospectivo entre 2009 y 2018. Se recogieron datos demográficos, clínicos, funcionales, radiológicos y de tratamiento.ResultadosDe un total de 225 pacientes, 11 presentaron SPE (prevalencia del 4,8%). Dos fueron excluidos. La edad media fue 39,33±16 años, 6 eran mujeres. Los síntomas principales fueron la disnea y el dolor pleurítico. La capacidad vital forzada media fue del 49%, la capacidad pulmonar total del 60%, la capacidad de difusión de monóxido de carbono del 66%, el factor de transferencia para el monóxido de carbono del 128%, la presión inspiratoria máxima del 66% y la presión espiratoria máxima del 82%. Todos los pacientes recibieron corticosteroides. Después de una mediana de seguimiento de 19 meses, 4 casos presentaron mejoría y 4 estabilización.ConclusionesEl SPE debe tenerse presente en todo paciente lúpico con disnea de causa no evidente. Si bien suele evolucionar con mejoría, la mayoría queda con deterioro persistente a pesar del tratamiento. (AU)

Introduction: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time.MethodsA retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected.ResultsOut of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable.ConclusionsSLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment. (AU)

[Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients]. / Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes.

INTRODUCTION: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time. METHODS: A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected. RESULTS: Out of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable. CONCLUSIONS: SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment.

Description of a single centre cohort of patients with systemic sclerosis from the University Hospital of Buenos Aires and factors associated with lung function deterioration. A retrospective study.

INTRODUCTION: Given the paucity of data in Latin America and especially in Argentina regarding the epidemiology of SSc, the prevalence of ILD, its course, and particularly the response to treatment, our objective was to evaluate a cohort of SSc patients evaluated in a single University Hospital in Buenos Aires. PATIENTS/METHODS: We included 152 patients with SSc, followed from disease onset to last pulmonary function test and with at least two PFT and up to 30 months between each. RESULTS: Sixty-one percent had diffuse SSc (DSSc) and 32% limited SSc (LSSc). The only significant clinical differences between these groups were a higher initial mRodnan score and prevalence of ILD in the DSSc. These also had significantly more anti Scl-70 (Topoisomerase 1) antibodies compared to the LSSC group who had significantly more anti centromere antibodies. The DSSc group also had significantly more extensive damage on HRCT with no differences in terms of imaging patterns. Comparing patients with and without ILD by HRCT, those with ILD had significantly more extensive damage, significantly more anti Scl-70 antibodies, and significantly fewer anti centromere antibodies than those without ILD. Patients whose ILD progressed had a smoking history (OR 4.97) and prior immunosuppressive treatment (OR 15.6) (multivariate analysis). Overall disease duration was significantly shorter in those who progressed. CONCLUSIONS: Our SSc population had similar characteristics to those described elsewhere as well as prevalence of ILD and its progression. We found a shorter disease duration, smoking, and prior immunosuppressive treatment to be associated with ILD progression.

Description of a single centre cohort of patients with systemic sclerosis from the University Hospital of Buenos Aires and factors associated with lung function deterioration. A retrospective study / Descripción de una cohorte de pacientes con esclerosis sistémica del Hospital de la Universidad de Buenos Aires y factores asociados al deterioro de la función pulmonar. Un estudio retrospectivo

Introduction: Given the paucity of data in Latin America and especially in Argentina regarding the epidemiology of SSc, the prevalence of ILD, its course, and particularly the response to treatment, our objective was to evaluate a cohort of SSc patients evaluated in a single University Hospital in Buenos Aires. Patients/Methods: We included 152 patients with SSc, followed from disease onset to last pulmonary function test and with at least two PFT and up to 30 months between each. Results: Sixty-one percent had diffuse SSc (DSSc) and 32% limited SSc (LSSc). The only significant clinical differences between these groups were a higher initial mRodnan score and prevalence of ILD in the DSSc. These also had significantly more anti Scl-70 (Topoisomerase 1) antibodies compared to the LSSC group who had significantly more anti centromere antibodies. The DSSc group also had significantly more extensive damage on HRCT with no differences in terms of imaging patterns. Comparing patients with and without ILD by HRCT, those with ILD had significantly more extensive damage, significantly more anti Scl-70 antibodies, and significantly fewer anti centromere antibodies than those without ILD. Patients whose ILD progressed had a smoking history (OR 4.97) and prior immunosuppressive treatment (OR 15.6) (multivariate analysis). Overall disease duration was significantly shorter in those who progressed. Conclusions: Our SSc population had similar characteristics to those described elsewhere as well as prevalence of ILD and its progression. We found a shorter disease duration, smoking, and prior immunosuppressive treatment to be associated with ILD progression.(AU)

Introducción: La escasez de datos en Latinoamérica, y especialmente en Argentina, sobre la epidemiología de la esclerosis sistémica (SSc), la prevalencia de enfermedad pulmonar intersticial (EPID) y su progresión, llevó a evaluar una cohorte de pacientes con SSc atendidos en un hospital universitario de Buenos Aires, Argentina. Pacientes/Métodos: Incluimos 152 pacientes con SSc, seguidos desde el inicio de la enfermedad hasta el último examen funcional respiratorio (EFR) y con por lo menos dos EFR separados por un mínimo de 30 meses. Resultados: El 61% tenían enfermedad difusa (DSSc) y el 32%, limitada (LSSc). Aquellos con DSSc tuvieron significativamente un mayor índice modificado de Rodnan y prevalencia de EPID. Estos también tuvieron significativamente más anticuerpos anti-Scl-70 (topoisomerasa 1) comparados con LSSc, quienes tuvieron significativamente más anticuerpos anti-centrómero. Aquellos con DSSc mostraron significativamente más daño en la tomografía computada de alta resolución (TACAR), pero sin diferencias respecto a patrón de imágenes. Aquellos con EPID por TACAR tuvieron significativamente más daño, más anticuerpos anti Scl-70 y menos anticuerpos anti-centrómero que aquellos sin EPID. La progresión de EPID (análisis multivariado) se relacionó con consumo de tabaco (OR: 4,97) y uso previo de inmunosupresores (OR: 15,6). La duración de la enfermedad fue menor en los que progresaron. Conclusiones:Nuestra población de SSc tuvo características similares a lo descripto en el resto del mundo, así como la prevalencia y la progresión de EPID. Encontramos una menor duración de enfermedad, el consumo de tabaco y el uso previo de inmunosupresores asociados a la progresión de EPID.(AU)

[Hypersensitivity pneumonitis. A series of nine cases with surgical lung biopsy]. / Neumonitis por hipersensibilidad. Serie de nueve casos con biopsia pulmonar quirúrgica.

In a series of nine patients with histopathological diagnosis of hypersensitivity pneumonitis, we retrospectively evaluated clinical data, exposure related factors, pulmonary function tests and chest computed tomography scans. A restrictive abnormality with reduction of diffusion capacity for carbon monoxide was mainly found. Chest scans showed fibrotic patterns in most cases; ground glass attenuation areas with mosaic pattern and consolidation in the rest. Exposure to avian antigens, cereal grains and air conditioners contaminated with fungi yeasts and bacteria, were suspected from clinical data in two-thirds of the cases. Since there are no unique features that allow differentiation from other interstitial lung diseases, a high clinical suspicion is required and a careful search of environmental exposure to possible antigens is needed that, together with clinical, radiological and pathological data, may lead to diagnosis.

Neumonitis por hipersensibilidad: Serie de nueve casos con biopsia pulmonar quirúrgica / Hypersensitivity pneumonitis. A series of nine cases with surgical lung biopsy

En una serie de nueve pacientes con diagnóstico histopatológico de neumonitis por hipersensibilidad, se revisaron en forma retrospectiva la historia clínica, los factores de exposición, las pruebas de función pulmonar y las tomografías computarizadas de tórax. La función pulmonar evidenció principalmente compromiso restrictivo con disminución de la difusión de monóxido de carbono. Las tomografías de tórax mostraron patrones fibróticos en la mayoría de los casos; áreas de vidrio esmerilado con patrón en mosaico y áreas de consolidación en el resto. Pudo detectarse exposición a antígenos aviarios, granos de cereales y acondicionadores de aire contaminados con esporas de hongos y bacterias, en dos tercios de los casos. Dado que no existen características únicas que permitan diferenciar la neumonitis por hipersensibilidad de otras enfermedades del intersticio pulmonar, se requiere de un alto índice de sospecha clínica y de una detallada búsqueda de exposición ambiental que deberán complementarse con los datos clínicos, radiológicos y de anatomía patológica para llegar al diagnóstico.

In a series of nine patients with histopathological diagnosis of hypersensitivity pneumonitis, we retrospectively evaluated clinical data, exposure related factors, pulmonary function tests and chest computed tomography scans. A restrictive abnormality with reduction of diffusion capacity for carbon monoxide was mainly found. Chest scans showed fibrotic patterns in most cases; ground glass attenuation areas with mosaic pattern and consolidation in the rest. Exposure to avian antigens, cereal grains and air conditioners contaminated with fungi yeasts and bacteria, were suspected from clinical data in two-thirds of the cases. Since there are no unique features that allow differentiation from other interstitial lung diseases, a high clinical suspicion is required and a careful search of environmental exposure to possible antigens is needed that, together with clinical, radiological and pathological data, may lead to diagnosis.