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Inicio Revista Colombiana de Reumatología Systemic sclerosis and interstitial lung disease: From pathogenesis, to screenin...
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Vol. 31. Issue S1.
Interstitial Lung Disease Associated with Autoimmune Diseases
Pages S54-S66 (April 2024)
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Vol. 31. Issue S1.
Interstitial Lung Disease Associated with Autoimmune Diseases
Pages S54-S66 (April 2024)
Review article
Systemic sclerosis and interstitial lung disease: From pathogenesis, to screening, diagnosis, and classification
Esclerosis sistémica y enfermedad pulmonar intersticial: de la patogenia al cribado, el diagnóstico y la clasificación
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Helena Codesa, Aslihan Avanoglu Gulerb,c, Corrado Campochiaroc, Marco Matucci Cerinicc, Ivan Castellvia,
Corresponding author
Castellvi@santpau.cat

Corresponding author.
a Department of Rheumatology, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain
b Department of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey
c Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
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Table 1. Radiological patterns and signs of fibrosis on chest high resolution computed tomography.
Table 2. Definition of progressive pulmonary fibrosis.
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Special issue
This article is part of special issue:
Vol. 31. Issue S1

Interstitial Lung Disease Associated with Autoimmune Diseases

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Abstract

Interstitial lung disease (ILD) is a common and potentially devastating complication of systemic sclerosis (SSc), a chronic autoimmune disorder characterized by fibrosis and vascular abnormalities. The association between SSc and ILD underscores the intricate interplay between immune dysregulation, vasculopathy, and tissue fibrosis. This review provides a comprehensive overview of the immunological, clinical, and radiological features of ILD in the context of SSc. It highlights the diverse spectrum of ILD patterns observed in SSc patients, ranging from non-specific interstitial pneumonia to usual interstitial pneumonia. The intricate pathogenic mechanisms linking SSc and ILD involve aberrant immune responses, endothelial dysfunction, profibrotic cytokine signaling, and genetic factors. Immunological alterations, diagnostic challenges, and prognostic implications are discussed, underscoring the need for multidisciplinary management strategies. By elucidating the complex relationship between SSc and ILD, this review aims to contribute to a deeper understanding of the underlying mechanisms and facilitate the development of interdisciplinary interventions for improved patient outcomes.

Keywords:
Systemic sclerosis
Interstitial lung disease
Lung
Treatment
Approach
Resumen

La enfermedad pulmonar intersticial (EPI) es una complicación frecuente y potencialmente devastadora de la esclerosis sistémica (SSc), un trastorno autoinmune crónico caracterizado por fibrosis y anomalías vasculares. La asociación entre la SSc y la EPI subraya la difícil interacción entre la desregulación inmunitaria, la vasculopatía y la fibrosis tisular. Esta revisión proporciona una visión global de las características inmunológicas, clínicas y radiológicas de la EPI en el contexto de la SSc; de igual manera, se destaca el diverso espectro de patrones de EPI observados en pacientes con SSc, que van desde la neumonía intersticial inespecífica a la habitual. Los intrincados mecanismos patogénicos que relacionan la SSc y la EPI implican respuestas inmunitarias aberrantes, disfunción endotelial, señalización profibrótica de citoquinas y factores genéticos. Se discuten las alteraciones inmunológicas, los retos diagnósticos y las repercusiones pronósticas, subrayando la necesidad de estrategias de gestión multidisciplinares. Al elucidar la compleja relación entre la SSc y la EPI, esta revisión pretende contribuir a una comprensión más profunda de los mecanismos subyacentes y facilitar el desarrollo de intervenciones interdisciplinarias para mejorar los resultados de los pacientes.

Palabras clave:
Esclerosis sistémica
Enfermedad intersticial pulmonar
Pulmón
Tratamiento
Abordaje

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