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• W2912831644 abstract "Introduction ILD represents a heterogeneous spectrum of subgroups, the biggest being IPF and connective tissue disease ILD (CTD-ILD). In IPF, a lack of biomarkers and the dangers of lung biopsy make the diagnosis dependant on HRCT. Usual Interstitial Pneumonia changes are typical of IPF, but can also occur in CTD-ILD, making misdiagnosis a possibility. While CTD-ILDs can respond to immunosuppression with corticosteroids and disease modifying drugs, these agents are ineffective and harmful in IPF, and therefore contraindicated (Raghu et al. N Eng J Med 2012; 366: 1968). Where possible, it is thus important to exclude a covert CTD-ILD in IPF patients. Previous studies have detected autoantibodies in 6%–38% of patients with idiopathic interstitial pneumonias (Song et al. Yonsei Med J 2015; 56: 676, Watanabe et al. Respir Med 2011; 105: 1238), but wide-ranging CTD serotyping has not been undertaken in a large UK IPF cohort. Methods The gold standard for autoantibody detection is immunoprecipitation (IP), which very occasionally detects anti-Ro, but no other, autoantibodies in normal subjects. IP of radio-labelled K562 cells was used to serotype 250 IPF patients recruited via UK-BILD. All satisfied the 2011 ATS/ERS diagnostic guidelines and had IPF-specific HRCT changes (Raghu et al. Am J Respir Crit Care Med 2011; 183: 788). Proteins recognised by autoantibodies from patients’ serum were fractionated by 9% SDS-PAGE gel, and identified by autoradiography. Known autoantigens were identified by direct comparison with antibody positive controls on the same gel. Results Four of 250 IPF patients (1.6%) were IP-positive for recognised CTD-specific autoantibodies, including one with an anti-synthetase (anti-OJ) and three with scleroderma or scleromyositis overlap-associated antibodies (anti-RNA Polymerase II, anti-PM-Scl and anti-Ku respectively). There were no clinical or radiographic characteristics which distinguished antibody positive from antibody negative cases. Conclusion Although CTD-specific autoantibodies were detected in only a tiny proportion of IPF patients, to suggest a possible misdiagnosis, this could have potentially serious therapeutic implications for individual patients. We recommend that, while IPF is clearly a robust diagnosis when made by multidisciplinary teams in tertiary centres, clinicians should endeavour to undertake comprehensive autoantibody testing in all IPF cases." @default.
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• W2912831644 date "2018-12-01" @default.
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• W2912831644 title "P152 Results from serotyping by immunoprecipitation suggest that a covert connective tissue disease (CTD) may be present in ~ 2% of ILD patients diagnosed with idiopathic pulmonary fibrosis (IPF)" @default.
• W2912831644 doi "https://doi.org/10.1136/thorax-2018-212555.310" @default.
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