Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody

A Sclafani,S B Montesi,A Sharma,J J Locascio,E M Miloslavsky,B P Little,K M D’Silva

doi:10.1186/s12931-019-1231-7

A Sclafani, S B Montesi + Show 5 more

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https://doi.org/10.1186/s12931-019-1231-7

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Abstract

BackgroundDistinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are well-recognized. However, the association between ILD and the myositis-associated antibodies, including anti-Ro52, is less established. Our objectives were to compare presenting phenotypes of patients with anti-Ro52 alone versus in combination with myositis-specific autoantibodies and to identify predictors of disease progression or death.MethodsWe performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes.ResultsThe majority of patients with ILD and anti-Ro52 had no established connective tissue disease (78%), and one-third had no rheumatologic symptoms. Thirteen patients (17.8%) required ICU admission for respiratory failure, with 84.6% all-cause mortality. Of the 73 subjects, 85.7% had a negative SS-A, and 49.3% met criteria for idiopathic pneumonia with autoimmune features (IPAF). The 50 patients with anti-Ro52 alone were indistinguishable from patients with anti-Ro52 plus a myositis-specific autoantibody. ICU admission was associated with poor outcomes (HR 12.97, 95% CI 5.07–34.0, p < 0.0001), whereas rheumatologic symptoms or ANA > = 1:320 were associated with better outcomes (HR 0.4, 95% CI 0.16–0.97, p = 0.04, and HR 0.29, 95% CI 0.09–0.81, p = 0.03, respectively).ConclusionsPresentations of ILD with the anti-Ro52 antibody are heterogeneous, and outcomes are similar when compared to anti-Ro52 plus myositis-specific antibodies. Testing for anti-Ro52 may help to phenotype unclassifiable ILD patients, particularly as part of the serologic criteria for IPAF. Further research is needed to investigate treatment of ILD in the setting of anti-Ro52 positivity.

Highlights

Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are well-recognized
The anti-SS-A 52 kD IgG, Sclafani et al Respiratory Research (2019) 20:256 is the most common autoantibody detected in patients with idiopathic inflammatory myopathies, and has been described to co-occur with myositis-specific antibodies, the anti-synthetase antibodies and antiMDA-5 [13,14,15,16,17,18]
Laboratory results including anti-Ro52 titer by enzyme immunoassay, antinuclear antibody (ANA), SS-A by enzyme-linked immunosorbent assay (ELISA), creatine kinase, aldolase, and myositis-specific autoantibodies including the anti-synthetase antibodies were collected

Summary

Introduction

Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are well-recognized. About the presentations of ILD and many of the myositis-associated autoantibodies frequently seen in myositis overlap syndromes. One such antibody, the anti-SS-A 52 kD IgG ( known as anti-Ro52), Sclafani et al Respiratory Research (2019) 20:256 is the most common autoantibody detected in patients with idiopathic inflammatory myopathies (estimated to be present in 20–30% of dermatomyositis/polymyositis), and has been described to co-occur with myositis-specific antibodies, the anti-synthetase antibodies and antiMDA-5 [13,14,15,16,17,18]. The coexistence of anti-Ro52 with myositis-specific autoantibodies is associated with an aggressive ILD course [7, 19, 20]

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