The Spectrum of Interstitial Lung Disease Associated with Autoimmune Diseases: Data of a 3.6-Year Prospective Study from a Referral Center of Interstitial Lung Disease and Lung Transplantation.

Belén Atienza-Mateo,Sonia Fernández-Rozas,Gerardo Blanco Rodríguez,Sara Remuzgo-Martínez,José Manuel Cifrián,Mónica Calderón-Goercke,Diana Prieto-Peña,Miguel Ángel González-Gay,David Iturbe-Fernández,José Javier Gómez-Román,Alfonso Corrales,Víctor Manuel Mora Cuesta

doi:10.3390/jcm9061606

Abstract

Interstitial lung disease (ILD) may occur in patients with a rheumatic autoimmune disease (AD), increasing their risk of morbidity and mortality. However, little is known about the prevalence of AD in patients diagnosed with an ILD. In this prospective study, we determined the spectrum of ILD associated with AD (AD-ILD) among patients sent for assessment to a single clinic of ILD and lung transplantation from a referral center between May 2016 and December 2019. ILD diagnosis was made by pneumologists based on clinical and radiological findings and pulmonary function test abnormalities. All patients with ILD were also assessed by experienced rheumatologists. During the period of assessment, 338 patients were diagnosed with ILD. Among them, 32.8% fulfilled definitions for an AD. Most cases with AD-ILD had a diagnosis of rheumatoid arthritis (27.0%), systemic sclerosis (26.1%) or anti-synthetase syndrome (17.1%). Interestingly, 18% of the patients with AD-ILD were diagnosed as having an interstitial pneumonia with autoimmune features. Antinuclear antibodies and non-specific interstitial pneumonia were the most frequent positive autoantibodies and radiological pattern found in AD-ILD patients, respectively. In conclusion, our study indicates that a high number of ILD patients have a related AD. Consequently, close collaboration among rheumatologists and pneumologists is needed.

Highlights

Interstitial lung diseases (ILDs) comprise a wide range of parenchymal lung disorders with a known possible cause (e.g., allergens, occupational exposures, pharmacotoxicity or rheumatic autoimmune diseases (ADs)) or with an unknown origin, called idiopathic interstitial pneumonias (IIPs)
Among the 338 ILD patients, 143 (42.3%) were diagnosed as having Idiopathic pulmonary fibrosis (IPF), 111 (32.8%) fulfilled definitions for an AD and 84 (24.9%) patients were classified as having an IIP-non IPF (49 with unclassifiable IIP, 23 with idiopathic non-specific interstitial pneumonia (NSIP), 5 with pleuroparenchymal fibroelastosis, 4 with respiratory bronchiolitis-ILD, 1 with desquamative interstitial pneumonia, 1 with idiopathic lymphoid interstitial pneumonia, and 1 with cryptogenic organizing pneumonia)
In this 3.6-year prospective study, we determined the frequency and the spectrum of the different types of associated ILD (AD-ILD) among 338 consecutive patients referred to our ILD multidisciplinary unit

Summary

Introduction

Interstitial lung diseases (ILDs) comprise a wide range of parenchymal lung disorders with a known possible cause (e.g., allergens, occupational exposures, pharmacotoxicity or rheumatic autoimmune diseases (ADs)) or with an unknown origin, called idiopathic interstitial pneumonias (IIPs). The latter are classified as major, rare or unclassifiable IIPs. Idiopathic pulmonary fibrosis (IPF) is one of the major chronic fibrosing IIP [1]. The prevalence of ILD in patients with an established AD varies from different reports and the particular AD. Little is known about the prevalence of AD in patients diagnosed with an ILD, being reported that approximately 30% of them fulfill criteria of an AD [12]

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