Rare Interstitial Lung Diseases: a Narrative Review

Overview

Journal J Thorac Dis

Publisher AME Publishing Company

Specialty Pulmonary Medicine

Date 2024 Oct 24

PMID 39444900

Authors

Karina Portillo Carroz

Blanca Urrutia-Royo

Antonio Marin

Laura Rodriguez Pons

Paloma Millan-Billi

Antoni Rosell

Onofre Moran-Mendoza

Affiliations

Soon will be listed here.

Abstract

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 review articles, meta-analyses, systematic reviews, case series, prospective studies, society guidelines, editorials in peer-reviewed journals; scientific books on the subject. 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.

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