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Pulmonary involvement in primary Sjögren’s Syndrome
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Introdução: A síndrome de Sjögren primária (SSp) é uma doença crónica imunomediada, caracterizada pela inflamação das glândulas exócrinas. Pode apresentar várias manifestações extraglandulares, entre as quais envolvimento pulmonar.
Objetivos: Estimar a frequência e o tipo de envolvimento pulmonar na SSp, identificar diferenças entre os doentes com e sem envolvimento pulmonar; caracterizar uma amostra de doentes com doença intersticial pulmonar (DIP) associada à SSp.
Métodos: Realizámos um estudo retrospetivo, numa amostra de doentes com o diagnóstico de SSp seguidos no Hospital Garcia de Orta e registados em Reuma.pt. Foram aplicados testes paramétricos e não paramétricos e os resultados foram considerados estatisticamente significativos para um valor p<0.05.
Resultados: Foram analisados 198 doentes. Destes, 19 (9.6%) apresentavam atingimento pulmonar. A DPI foi reportada em onze doentes (5.6%) e a presença de bronquiectasias em oito doentes (4%). Um doente com bronquiectasias apresentava bronquiolite folicular concomitante. Os doentes com envolvimento pulmonar eram mais velhos (63.2 ± 9.3 anos vs. 55.2 ± 13.3 anos, valor p 0.01), tinham maior duração de SSp (8.69 [4.75-13] years vs. 5.5 [IQR 2-9] years, valor p 0.023) e uma maior tendência a serem fumadores (21.1% vs. 11.2%, valor p 0.05).
Em 36.3% dos pacientes com DPI, o diagnóstico de doença pulmonar precedeu o diagnóstico de SSp. A pneumonia intersticial inespecífica foi o padrão de DPI mais prevalente (63.7%), seguida da pneumonia intersticial linfocítica (36.4%). Fármacos imunossupressores foram utilizados em seis (54.5%) doentes com DPI, sendo os antifibróticos associados em metade deles.
Conclusões: O envolvimento pulmonar foi reportado em 9.6% dos doentes da nossa coorte, sendo a DPI a apresentação mais frequente, seguida de bronquiectasias. Uma idade mais avançada, uma maior duração da SSp e o tabagismo parecem estar associados ao desenvolvimento de doença pulmonar.
Introduction: Primary Sjögren's syndrome (pSS) is a chronic, immune-mediated disease mainly characterized by inflammation of the exocrine glands. It can present with several extra-glandular manifestations, among which pulmonary involvement is included. Objectives: We aim to estimate the frequency and type of pulmonary involvement in pSS, identify differences between patients with and without pulmonary involvement and characterize patients with pSS-interstitial lung disease (ILD). Methods: We conducted a retrospective study in a cohort of patients with clinical diagnosis of pSS, followed at Hospital Garcia de Orta, and registered in Reuma.pt. Parametric and nonparametric tests were applied, and the results were considered statically significant for a p-value<0.05. Results: We analyzed 198 patients. From these, 19 (9.6%) had lung involvement. ILD was reported in eleven patients (5.6%) and bronchiectasis in eight patients (4%). One patient with bronchiectasis had concomitant follicular bronchiolitis. Patients with pulmonary involvement were older (63.2 ± 9.3 years vs. 55.2 ± 13.3 years, p-value 0.01), had a longer pSS duration (8.69 [4.75-13] years vs. 5.5 [IQR 2-9] years, p-value 0.023) and were more likely to be smokers (21.1% vs. 11.2%, p-value 0.05). In 36.3% of ILD patients, the diagnosis of lung disease preceded the diagnosis of pSS. Nonspecific interstitial pneumonia was the most prevalent ILD pattern (63.7%), followed by lymphocytic interstitial pneumonia (36.4%). Immunosuppressive drugs were used in six (54.5%) ILD patients, with antifibrotic being associated in half of them. Conclusions: Pulmonary involvement was reported in 9.6% of patients in our cohort, with ILD accounting for the most frequent presentation, followed by bronchiectasis. An older age, a longer pSS duration and smoking seem to be associated with the development of lung disease.
Introduction: Primary Sjögren's syndrome (pSS) is a chronic, immune-mediated disease mainly characterized by inflammation of the exocrine glands. It can present with several extra-glandular manifestations, among which pulmonary involvement is included. Objectives: We aim to estimate the frequency and type of pulmonary involvement in pSS, identify differences between patients with and without pulmonary involvement and characterize patients with pSS-interstitial lung disease (ILD). Methods: We conducted a retrospective study in a cohort of patients with clinical diagnosis of pSS, followed at Hospital Garcia de Orta, and registered in Reuma.pt. Parametric and nonparametric tests were applied, and the results were considered statically significant for a p-value<0.05. Results: We analyzed 198 patients. From these, 19 (9.6%) had lung involvement. ILD was reported in eleven patients (5.6%) and bronchiectasis in eight patients (4%). One patient with bronchiectasis had concomitant follicular bronchiolitis. Patients with pulmonary involvement were older (63.2 ± 9.3 years vs. 55.2 ± 13.3 years, p-value 0.01), had a longer pSS duration (8.69 [4.75-13] years vs. 5.5 [IQR 2-9] years, p-value 0.023) and were more likely to be smokers (21.1% vs. 11.2%, p-value 0.05). In 36.3% of ILD patients, the diagnosis of lung disease preceded the diagnosis of pSS. Nonspecific interstitial pneumonia was the most prevalent ILD pattern (63.7%), followed by lymphocytic interstitial pneumonia (36.4%). Immunosuppressive drugs were used in six (54.5%) ILD patients, with antifibrotic being associated in half of them. Conclusions: Pulmonary involvement was reported in 9.6% of patients in our cohort, with ILD accounting for the most frequent presentation, followed by bronchiectasis. An older age, a longer pSS duration and smoking seem to be associated with the development of lung disease.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2024
Palavras-chave
Síndrome de Sjögren Envolvimento pulmonar Doença do interstício pulmonar Reumatologia