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

Background Distinct 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 o...

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Published inRespiratory research Vol. 20; no. 1; pp. 256 - 9
Main Authors Sclafani, A., D’Silva, K. M., Little, B. P., Miloslavsky, E. M., Locascio, J. J., Sharma, A., Montesi, S. B.
Format Journal Article
LanguageEnglish
Published London BioMed Central 12.11.2019
BioMed Central Ltd
BMC
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ISSN1465-993X
1465-9921
1465-993X
DOI10.1186/s12931-019-1231-7

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Abstract Background Distinct 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. Methods We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. Results The 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). Conclusions Presentations 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.
AbstractList Abstract Background Distinct 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. Methods We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. Results The 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). Conclusions Presentations 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.
Distinct 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.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.We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes.METHODSWe performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes.The 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).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).Presentations 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.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.
Distinct 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. We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. The 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). Presentations 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.
Background Distinct 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. Methods We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. Results The 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). Conclusions Presentations 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. Keywords: Interstitial lung disease, Myositis, Antibodies, Interstitial pneumonia with autoimmune features, Connective tissue disease
Background Distinct 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. Methods We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. Results The 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). Conclusions Presentations 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.
Distinct 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. We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. The 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). Presentations 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.
ArticleNumber 256
Audience Academic
Author Locascio, J. J.
Miloslavsky, E. M.
Sclafani, A.
Montesi, S. B.
D’Silva, K. M.
Sharma, A.
Little, B. P.
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Issue 1
Keywords Antibodies
Connective tissue disease
Myositis
Interstitial lung disease
Interstitial pneumonia with autoimmune features
Language English
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Snippet Background Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are...
Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are...
Background Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are...
Abstract Background Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase...
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StartPage 256
SubjectTerms Adult
Aged
Aged, 80 and over
Antibodies
Autoantibodies
Autoantibodies - blood
Autoimmunity
Cohort Studies
Connective tissue disease
Development and progression
Female
Follow-Up Studies
Humans
Intensive Care Units - trends
Interstitial lung disease
Interstitial pneumonia with autoimmune features
Ligases
Lung diseases
Lung Diseases, Interstitial - blood
Lung Diseases, Interstitial - diagnostic imaging
Male
Medicine
Medicine & Public Health
Middle Aged
Mortality
Myositis
Patient Admission - trends
Phenotypes
Pneumology/Respiratory System
Pneumonia
Pulmonary fibrosis
Respiratory insufficiency
Respiratory tract diseases
Retrospective Studies
Ribonucleoproteins - blood
Treatment Outcome
Young Adult
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Title Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody
URI https://link.springer.com/article/10.1186/s12931-019-1231-7
https://www.ncbi.nlm.nih.gov/pubmed/31718649
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