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J. Clin. Med. 2017, 6(5), 51; doi:10.3390/jcm6050051

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)

1
Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, NSW 2050, Australia
2
Sydney Medical School, University of Sydney, Sydney, NSW 2006, Australia
3
Immunopathology Laboratory, Southwest Sydney Pathology Service, Sydney, NSW 2050, Australia
4
Department of Clinical Immunology and Allergy, Royal Prince Alfred Hospital, Sydney, NSW 2050, Australia
5
Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, NSW 2050, Australia
6
Department of Respiratory, Princess Alexandra Hospital, Woolloongabba, QLD 4102, Australia
7
Department of Rheumatology, Monash Health, Clayton, VIC 3168, Australia
8
Department Medicine, Monash University, Clayton, VIC 3168, Australia
*
Author to whom correspondence should be addressed.
Academic Editor: Paul Huang
Received: 5 March 2017 / Revised: 20 April 2017 / Accepted: 29 April 2017 / Published: 4 May 2017
(This article belongs to the Special Issue Chronic Respiratory Diseases)
View Full-Text   |   Download PDF [476 KB, uploaded 4 May 2017]   |  

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. View Full-Text
Keywords: interstitial lung disease; connective tissue disease; autoantibodies; interstitial pneumonia with autoimmune features; diagnosis interstitial lung disease; connective tissue disease; autoantibodies; interstitial pneumonia with autoimmune features; diagnosis
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MDPI and ACS Style

Jee, A.S.; Adelstein, S.; Bleasel, J.; Keir, G.J.; Nguyen, M.; Sahhar, J.; Youssef, P.; Corte, T.J. Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF). J. Clin. Med. 2017, 6, 51.

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