PMID- 33754221
OWN - NLM
STAT- MEDLINE
DCOM- 20210518
LR  - 20210518
IS  - 1434-9949 (Electronic)
IS  - 0770-3198 (Linking)
VI  - 40
IP  - 6
DP  - 2021 Jun
TI  - Risk of progression of idiopathic pulmonary fibrosis to connective tissue 
      disease: a long-term observational study in 527 patients.
PG  - 2447-2456
LID - 10.1007/s10067-021-05659-x [doi]
AB  - OBJECTIVE: Connective tissue disease (CTD) might occur during the course of 
      idiopathic pulmonary fibrosis (IPF). Clinical factors associated with CTD 
      development in IPF patients have still not been identified. We investigated which 
      antibodies have a significant association with the development of CTD during the 
      clinical course of IPF. METHODS: We retrospectively reviewed the records of 527 
      patients with a first diagnosis of IPF between January 2007 and March 2014 and 
      investigated the time to CTD development after IPF diagnosis in these patients. 
      RESULTS: CTD developed in 15 patients at a median of 2.1 years (range 1.2-4.8) 
      after IPF diagnosis. All patients had anti-neutrophil cytoplasmic antibodies 
      (ANCA) or autoantibodies that met the serology criteria for interstitial 
      pneumonia with autoimmune features (IPAF). Survival duration for IPF patients 
      with progression to CTD was 5.3 (3.8, 6.7) years, which was significantly longer 
      than for IPF patients without progression to CTD [2.9 (1.7, 4.8), p = 0.001]. 
      Independent risk factors for CTD development in IPF patients included female 
      gender [adjusted hazard ratio (HR) 5.319, p = 0.0082], titer of rheumatoid factor 
      (RF; adjusted HR, 1.006; p = 0.022), titer of anti-citrullinated protein antibody 
      (ACPA; adjusted HR, 1.009; p = 0.0011), and titer of myeloperoxidase (MPO)-ANCA 
      (adjusted HR, 1.02; p < 0.0001). CONCLUSION: Progression to CTD is uncommon in 
      IPF patients. However, a significant number of IPF patients with high titers of 
      RF, ACPA, or MPO-ANCA progressed to CTD. RF, ACPA, and MPO-ANCA might be 
      significantly associated with CTD development in IPF patients. Key Points * A 
      significant number of IPF patients with high titers of RF, ACPA, or MPO-ANCA 
      progressed to CTD. * IPF/UIP with high titers of RF, ACPA, or MPO-ANCA might be 
      the initial clinical manifestation of CTD. * RF, ACPA, and MPO-ANCA may be 
      significantly associated with the development of pulmonary fibrosis in patients 
      with CTD.
FAU - Ghang, Byeongzu
AU  - Ghang B
AD  - Division of Rheumatology, Jeju National University School of Medicine, Jeju 
      National University Hospital, Jeju, Republic of Korea.
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Nam, So Hye
AU  - Nam SH
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Lee, Jungsun
AU  - Lee J
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Lim, Doo-Ho
AU  - Lim DH
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Ulsan 
      University Hospital, Ulsan, Republic of Korea.
FAU - Ahn, Soo Min
AU  - Ahn SM
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Oh, Ji Seon
AU  - Oh JS
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Hong, Seokchan
AU  - Hong S
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Kim, Yong-Gil
AU  - Kim YG
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Yoo, Bin
AU  - Yoo B
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
FAU - Kim, Jinseok
AU  - Kim J
AD  - Division of Rheumatology, Jeju National University School of Medicine, Jeju 
      National University Hospital, Jeju, Republic of Korea.
FAU - Lee, Chang-Keun
AU  - Lee CK
AUID- ORCID: 0000-0003-3653-7515
AD  - Division of Rheumatology, University of Ulsan College of Medicine, Asan Medical 
      Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea. 
      cklee@amc.seoul.kr.
LA  - eng
PT  - Journal Article
PT  - Observational Study
DEP - 20210322
PL  - Germany
TA  - Clin Rheumatol
JT  - Clinical rheumatology
JID - 8211469
RN  - EC 1.11.1.7 (Peroxidase)
SB  - IM
EIN - Clin Rheumatol. 2021 Apr 15;:. PMID: 33856625
MH  - *Connective Tissue Diseases/complications
MH  - Female
MH  - Humans
MH  - *Idiopathic Pulmonary Fibrosis/complications
MH  - Peroxidase
MH  - Retrospective Studies
MH  - Tomography, X-Ray Computed
OTO - NOTNLM
OT  - Autoantibodies
OT  - Autoimmunity
OT  - Connective tissue diseases
OT  - Idiopathic pulmonary fibrosis
EDAT- 2021/03/24 06:00
MHDA- 2021/05/19 06:00
CRDT- 2021/03/23 07:01
PHST- 2020/10/13 00:00 [received]
PHST- 2021/02/16 00:00 [accepted]
PHST- 2021/02/15 00:00 [revised]
PHST- 2021/03/24 06:00 [pubmed]
PHST- 2021/05/19 06:00 [medline]
PHST- 2021/03/23 07:01 [entrez]
AID - 10.1007/s10067-021-05659-x [pii]
AID - 10.1007/s10067-021-05659-x [doi]
PST - ppublish
SO  - Clin Rheumatol. 2021 Jun;40(6):2447-2456. doi: 10.1007/s10067-021-05659-x. Epub 
      2021 Mar 22.