RESUMO
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.
Assuntos
Alveolite Alérgica Extrínseca/diagnóstico , Idoso , Alveolite Alérgica Extrínseca/patologia , Alveolite Alérgica Extrínseca/cirurgia , Biópsia/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Doenças do Sistema Digestório , Pneumopatias , Lúpus Eritematoso Sistêmico , Doenças Musculares , Humanos , Feminino , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Masculino , Estudos Retrospectivos , Monóxido de Carbono/uso terapêutico , Síndrome , Pneumopatias/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Dispneia/etiologia , Pulmão/diagnóstico por imagemRESUMO
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.
Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Humanos , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , Imunossupressores , Pulmão , HospitaisRESUMO
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.