Abstract
The diagnosis of interstitial lung disease (ILD) requires meticulous evaluation for an underlying connective tissue disease (CTD), with major implications for prognosis and management. CTD associated ILD (CTD-ILD) occurs most commonly in the context of an established CTD, but can be the first and/or only manifestation of an occult CTD or occur in patients who have features suggestive of an autoimmune process, but not meeting diagnostic criteria for a defined CTD—recently defined as “interstitial pneumonia with autoimmune features” (IPAF). The detection of specific autoantibodies serves a critical role in the diagnosis of CTD-ILD, but there remains a lack of data to guide clinical practice including which autoantibodies should be tested on initial assessment and when or in whom serial testing should be performed. The implications of detecting autoantibodies in patients with IPAF on disease behaviour and management remain unknown. The evaluation of CTD-ILD is challenging due to the heterogeneity of presentations and types of CTD and ILD that may be encountered, and thus it is imperative that immunologic tests are interpreted in conjunction with a detailed rheumatologic history and examination and multidisciplinary collaboration between respiratory physicians, rheumatologists, immunologists, radiologists and pathologists.
Highlights
Interstitial lung diseases (ILDs) encompass chronic lung disorders characterised by damage to lung tissue by inflammation and/or fibrosis
This review summarises the literature regarding the utility of connective tissue disease (CTD)-associated autoantibodies in the diagnosis of CTD associated ILD (CTD-ILD), potential implications for ILD patients with positive autoantibodies but without full diagnostic features of a CTD, and current gaps in the available evidence
Immunologic tests serve an important role detecting potentially unrecognised CTD in ILD patients, especially when other dermatologic, arthritic or myopathic features are subtle or absent, but there is no consensus about which serological tests to obtain at first encounter
Summary
Interstitial lung diseases (ILDs) encompass chronic lung disorders characterised by damage to lung tissue by inflammation and/or fibrosis. The diagnosis of CTD associated ILD (CTD-ILD) is challenging and requires careful evaluation of clinical, physiological, radiological, histopathological and immunological markers of disease. For these reasons, a multidisciplinary approach has become the gold standard in ILD diagnosis, involving close communication between pulmonologists, rheumatologists, immunologists, radiologists and pathologists in a dynamic process that may require repeating as a patient’s disease evolves [11]. This review summarises the literature regarding the utility of CTD-associated autoantibodies in the diagnosis of CTD-ILD, potential implications for ILD patients with positive autoantibodies but without full diagnostic features of a CTD, and current gaps in the available evidence. Autoantibodies have demonstrable utility in the diagnosis and management of patients with CTD-ILD, there remains a lack of data to guide clinical practice
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