Rare interstitial lung diseases : a narrative review
Portillo, Karina (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Urrutia Royo, Blanca (Consorci Sanitari del Maresme)
Marin, Antonio (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Rodriguez Pons, Laura (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Millán-Billi, Paloma (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Rosell Gratacos, Antoni (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Moran-Mendoza, Onofre (Queen's University)
Universitat Autònoma de Barcelona

Fecha:	2024
Resumen:	Background and Objective: Interstitial lung diseases (ILDs) encompass over 200 entities. Among them, fibrosing lung diseases, have recently generated special interest due to the emerging therapies for their management. However, it is important to deepen our knowledge of other less prevalent ILD, since many of them are associated with a poor prognosis. This narrative review aims to provide a practical and up-to-date description of some poorly recognized ILD. It covers rare idiopathic interstitial pneumonias and their histologic patterns, genetic disorders with interstitial lung involvement (Hermansky-Pudlak syndrome), and ILD associated with benign proliferation of pulmonary lymphoid tissue, namely follicular bronchiolitis and granulomatous-lymphocytic interstitial lung disease. Methods: Electronic searches of PubMed and Google Scholar using specific keywords were conducted. Articles underwent screening for relevance, covering case reports, case series, observational cohorts, retrospective studies, meta-analysis, and review articles. The data included was limited to English and Spanish publications. Key Content and Findings: Despite the low prevalence of these diseases, the increased recognition of radiological patterns, pathological features, and diagnostic procedures, have permitted their better characterization. This review highlights epidemiology, clinical presentation, diagnosis, natural history, and treatment. Conclusions: Lesser-studied ILD represent a diagnostic and therapeutic challenge and can be frequently misdiagnosed. Also, due to the lack of randomized controlled trials, there are no well-established therapeutic options. Further studies or registries are needed to improve accurate diagnosis and management.
Derechos:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, i la comunicació pública de l'obra, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original. No es permet la creació d'obres derivades.
Lengua:	Anglès
Documento:	Article de revisió ; recerca ; Versió publicada
Materia:	Hermansky-Pudlak syndrome (HPS) ; Rare idiopathic interstitial pneumonias ; Acute fibrinous organizing pneumonia (AFOP) ; Granulomatous-lymphocytic interstitial lung disease (GLILD)
Publicado en:	Journal of Thoracic Disease, Vol. 16 Núm. 9 (30 2024) , p. 6320-6338, ISSN 2077-6624

DOI: 10.21037/jtd-24-450
PMID: 39444900

19 p, 1.8 MB

El registro aparece en las colecciones:
Documentos de investigación > Documentos de los grupos de investigación de la UAB > Centros y grupos de investigación (producción científica) > Ciencias de la salud y biociencias > Institut d'Investigació en Ciencies de la Salut Germans Trias i Pujol (IGTP)
Artículos > Artículos de investigación
Artículos > Artículos publicados