Progressive pulmonary fibrosis in systemic autoimmune diseases. A real life study.

INTRODUCTION: Interstitial lung diseases associated with systemic autoimmune diseases (ILD-SAD) can progress to a fibrotic form that can benefit from antifibrotic treatment. The aim of the study is to describe a cohort of patients with ILD-SAD who manifest progressive pulmonary fibrosis treated with antifibrotics. METHODS: Single-centre retrospective observational study from a tertiary care hospital on a cohort of patients with ILD-SAD with progressive pulmonary fibrosis evaluated in a joint pulmonology and rheumatology clinic that initiated treatment with antifibrotic drugs between 01/01/2019 and 01/12/2021. Clinical characteristics were analysed. The evolution of pulmonary function test and adverse effects during treatment were described. RESULTS: 18 patients were included. The mean age was 66.7 ±â€¯12.7 years, with a higher frequency of females (66.7%). Systemic sclerosis (SS) was the most frequent systemic autoimmune disease (36.8%). The majority of patients were receiving systemic glucocorticoid treatment (88.9%), 72.2% of patients were receiving treatment with disease-modifying drugs, the most frequent being mycophenolate mofetil (38.9%), and 22.2% with rituximab. Functional stability was observed after the start of antifibrotic treatment. Two patients died during follow-up, one due to progression of ILD. CONCLUSION: Our study suggests a beneficial effect of antifibrotic treatment added to immunomodulatory treatment in patients with fibrotic ILD-SAD in real life. In our cohort, patients with ILD-SAD with progressive fibrosing involvement show functional stability after starting antifibrotic treatment. Treatment tolerance was relatively good with a side effect profile similar to that described in the medical literature.

Fibrosis pulmonar en enfermedades autoinmunes sistémicas. Un estudio en vida real / Progressive pulmonary fibrosis in systemic autoimmune diseases. A real life study

Introducción: Las enfermedades pulmonares intersticiales difusas asociadas a enfermedades autoinmunes sistémicas (EPID-EAS) pueden presentar una progresión fibrótica. El objetivo principal del estudio es describir una serie de casos de pacientes con EPID-EAS que cursan con fibrosis pulmonar progresiva e inician tratamiento con fármacos antifibróticos. Métodos: Estudio observacional retrospectivo unicéntrico de un hospital de tercer nivel sobre una serie de casos de pacientes con EPID-EAS con fibrosis pulmonar progresiva valorados en una consulta conjunta de neumología y reumatología, que iniciaron tratamiento con fármacos antifibróticos entre el 01/01/2019 y el 01/12/2021. Se analizaron las características epidemiológicas, clínicas, funcionales, radiológicas y terapéuticas al inicio del tratamiento, y la evolución funcional durante el tratamiento, así como los efectos adversos. Resultados: Se incluyeron 18 pacientes. La edad media observada fue de 66,7±12,7 años, con mayor frecuencia de sexo femenino (66,7%), siendo la esclerosis sistémica la enfermedad autoinmune sistémica más frecuente (36,8%). La mayoría de los pacientes se encontraban en tratamiento con glucocorticoides sistémicos (88,9%), un 72,2% de pacientes con fármacos modificadores de la enfermedad, siendo el más frecuente el micofenolato mofetilo (38,9%), y un 22,2% con rituximab. Se observó una estabilidad funcional tras el inicio del tratamiento antifibrótico. Fallecieron 2 pacientes durante el seguimiento, uno de ellos como consecuencia de la progresión de la enfermedad intersticial pulmonar. Conclusión: Nuestro estudio sugiere un efecto beneficioso del tratamiento antifibrótico añadido al tratamiento inmunomodulador en pacientes con EPID-EAS fibrótica en vida real. En nuestra serie de casos, los pacientes con EPID-EAS con afectación fibrosante progresiva muestran una estabilidad funcional tras el inicio del tratamiento antifibrótico...(AU)

Introduction: Interstitial lung diseases associated with systemic autoimmune diseases (ILD-SAD) can progress to a fibrotic form that can benefit from antifibrotic treatment. The aim of the study is to describe a cohort of patients with ILD-SAD who manifest progressive pulmonary fibrosis treated with antifibrotics. Methods: Single-centre retrospective observational study from a tertiary care hospital on a cohort of patients with ILD-SAD with progressive pulmonary fibrosis evaluated in a joint pulmonology and rheumatology clinic that initiated treatment with antifibrotic drugs between 01/01/2019 and 01/12/2021. Clinical characteristics were analysed. The evolution of pulmonary function test and adverse effects during treatment were described. Results: 18 patients were included. The mean age was 66.7±12.7 years, with a higher frequency of females (66.7%). Systemic sclerosis (SS) was the most frequent systemic autoimmune disease (36.8%). The majority of patients were receiving systemic glucocorticoid treatment (88.9%), 72.2% of patients were receiving treatment with disease-modifying drugs, the most frequent being mycophenolate mofetil (38.9%), and 22.2% with rituximab. Functional stability was observed after the start of antifibrotic treatment. Two patients died during follow-up, one due to progression of ILD. Conclusion: Our study suggests a beneficial effect of antifibrotic treatment added to immunomodulatory treatment in patients with fibrotic ILD-SAD in real life. In our cohort, patients with ILD-SAD with progressive fibrosing involvement show functional stability after starting antifibrotic treatment. Treatment tolerance was relatively good with a side effect profile similar to that described in the medical literature.(AU)

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Síndrome de Richter con extensa afectación extranodal pleural aislada: importancia de la PET/TC / Richter Syndrome With Extensive Isolated Pleural Extranodal Involvement: The Importance of PET/CT Imaging

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Hemoptisis amenazante secundaria a aneurisma de Rasmussen en paciente VIH / Life-threatening hemoptysis secondary to Rasmussen's aneurysm in an HIV patient

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