PMID- 31718649
OWN - NLM
STAT- MEDLINE
DCOM- 20200429
LR  - 20200429
IS  - 1465-993X (Electronic)
IS  - 1465-9921 (Print)
IS  - 1465-9921 (Linking)
VI  - 20
IP  - 1
DP  - 2019 Nov 12
TI  - Presentations and outcomes of interstitial lung disease and the anti-Ro52 
      autoantibody.
PG  - 256
LID - 10.1186/s12931-019-1231-7 [doi]
LID - 256
AB  - 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.
FAU - Sclafani, A
AU  - Sclafani A
AUID- ORCID: 0000-0001-8229-8266
AD  - Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital 
      and Harvard Medical School, 100 Blossom St, Cox 201, Boston, MA, 02114, USA. 
      asclafani1@partners.org.
FAU - D'Silva, K M
AU  - D'Silva KM
AD  - Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital 
      and Harvard Medical School, Boston, MA, USA.
FAU - Little, B P
AU  - Little BP
AD  - Department of Radiology, Massachusetts General Hospital and Harvard Medical 
      School, Boston, MA, USA.
FAU - Miloslavsky, E M
AU  - Miloslavsky EM
AD  - Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital 
      and Harvard Medical School, Boston, MA, USA.
FAU - Locascio, J J
AU  - Locascio JJ
AD  - Biostatistics Center and Department of Neurology, Massachusetts General Hospital 
      and Harvard Medical School, Boston, MA, USA.
FAU - Sharma, A
AU  - Sharma A
AD  - Department of Radiology, Massachusetts General Hospital and Harvard Medical 
      School, Boston, MA, USA.
FAU - Montesi, S B
AU  - Montesi SB
AD  - Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital 
      and Harvard Medical School, 100 Blossom St, Cox 201, Boston, MA, 02114, USA.
LA  - eng
GR  - T32 AR007258/AR/NIAMS NIH HHS/United States
PT  - Journal Article
DEP - 20191112
PL  - England
TA  - Respir Res
JT  - Respiratory research
JID - 101090633
RN  - 0 (Autoantibodies)
RN  - 0 (Ribonucleoproteins)
RN  - 0 (SS-A antigen)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Autoantibodies/*blood
MH  - Cohort Studies
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Intensive Care Units/trends
MH  - Lung Diseases, Interstitial/*blood/*diagnostic imaging
MH  - Male
MH  - Middle Aged
MH  - Patient Admission/trends
MH  - Retrospective Studies
MH  - Ribonucleoproteins/*blood
MH  - Treatment Outcome
MH  - Young Adult
PMC - PMC6852961
OTO - NOTNLM
OT  - Antibodies
OT  - Connective tissue disease
OT  - Interstitial lung disease
OT  - Interstitial pneumonia with autoimmune features
OT  - Myositis
COIS- BPL is an academic author for Elsevier and receives royalties for his work. ASh 
      is Principal Investigator for the Hummingbird Inc. study (ClinicalTrials.gov 
      Identifier: NCT03452514). SBM is supported by grants from the Parker B Francis 
      Foundation and Scleroderma Foundation. SBM receives clinical trial support from 
      United Therapeutics paid to her institution. The remaining authors declare no 
      relevant financial disclosures or conflicts of interest.
EDAT- 2019/11/14 06:00
MHDA- 2020/04/30 06:00
PMCR- 2019/11/12
CRDT- 2019/11/14 06:00
PHST- 2019/08/07 00:00 [received]
PHST- 2019/11/01 00:00 [accepted]
PHST- 2019/11/14 06:00 [entrez]
PHST- 2019/11/14 06:00 [pubmed]
PHST- 2020/04/30 06:00 [medline]
PHST- 2019/11/12 00:00 [pmc-release]
AID - 10.1186/s12931-019-1231-7 [pii]
AID - 1231 [pii]
AID - 10.1186/s12931-019-1231-7 [doi]
PST - epublish
SO  - Respir Res. 2019 Nov 12;20(1):256. doi: 10.1186/s12931-019-1231-7.