Idiopathic and immune‐related pulmonary fibrosis: diagnostic and therapeutic challenges

Abstract

Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune‐related pulmonary fibrosis.

Recent advances in the diagnosis and management of interstitial pneumonia with autoimmune features: the perspective of rheumatologists

“…As included in the nomenclature for IPAF, patients with IPAF are assumed to have both IIP and ARD-ILD features, which are represented by idiopathic fibrosis and the autoimmunemediated inflammatory process ( Figure 1). Several genetic polymorphisms are known to be involved in the pathogenesis of ILD [36,37]. Idiopathic pulmonary fibrosis (IPF)associated gene mutations are found in patients with telomerase/telomerase-, surfactant protein-, immune function-, and mucin 5B (MUC5B)-related conditions [37].…”

“…Several genetic polymorphisms are known to be involved in the pathogenesis of ILD [36,37]. Idiopathic pulmonary fibrosis (IPF)associated gene mutations are found in patients with telomerase/telomerase-, surfactant protein-, immune function-, and mucin 5B (MUC5B)-related conditions [37]. Newton et al demonstrated that the leukocyte telomere length is longer in patients with IPAF and ARD-ILD than in those with IPF [38].…”

Prevalence of Novel Myositis Autoantibodies in a Large Cohort of Patients with Interstitial Lung Disease

“…Interstitial lung diseases (ILDs) are a heterogeneous group of diffuse parenchymal lung disorders, characterized by inflammation or fibrosis of the pulmonary interstitium. ILDs can be idiopathic or secondary to known causes including connective tissue diseases (CTDs) [1][2][3][4][5]. It is challenging to distinguish CTD-ILD from other ILDs as clinical, functional, radiological, and pathological characteristics can be similar [6].…”

“…Serologic testing for autoantibodies by a myositis blot is recommended in pulmonary fibrosis suspected for an underlying CTD, which includes myositis specific antibodies (MSA) and myositis associated antibodies (MAA) [ 1 , 3 , 4 , 6 , 11 , 12 , 13 , 14 , 15 , 16 ]. MSA and MAA are found in patients with idiopathic interstitial myopathies but also occur in patients with rheumatic diseases including CTD-ILDs [ 3 , 4 , 5 , 6 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ].…”

Immunopathobiology of chronic lung disease

“…The review by McLean‐Tooke and colleagues examines the challenges associated with the accurate diagnosis of ILDs in patients 1 . There are over 200 different entities of ILDs that have been described.…”