PMID- 34449068
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220809
IS  - 2198-6576 (Print)
IS  - 2198-6584 (Electronic)
IS  - 2198-6576 (Linking)
VI  - 8
IP  - 4
DP  - 2021 Dec
TI  - A Closer Look at the Role of Anti-CCP Antibodies in the Pathogenesis of 
      Rheumatoid Arthritis-Associated Interstitial Lung Disease and Bronchiectasis.
PG  - 1463-1475
LID - 10.1007/s40744-021-00362-4 [doi]
AB  - Rheumatoid arthritis (RA) is an articular disease with extra-articular 
      manifestations. Pulmonary manifestations are not uncommon and can involve all 
      compartments of the lungs with airway disease in the form of bronchiectasis or 
      bronchiolitis, interstitial lung disease (ILD), pleural effusions and parenchymal 
      lung nodules. The pulmonary features may present synchronously or after the 
      articular disease, but, importantly, it may be the first presentation in 10% of 
      patients in the absence of articular symptoms. Here we discuss the pathogenesis 
      of RA lung involvement, particularly interstitial lung disease and 
      bronchiectasis, focusing on the role anti-CCP antibodies (ACPAs). We highlight 
      the complex interplay among genetic, environmental and immune factors. 
      Furthermore, we explore the relationship of citrullination and smoking as well as 
      the concept of interstitial pneumonia with autoimmune features (IPAF), where 
      patients do not have evidence of another known cause of interstitial pneumonia 
      and have incomplete features of connective tissue disease (CTD). We surmise that 
      the frequency and titers of rheumatoid factor (RF) and ACPAs are increased in 
      bronchiectasis and RA-bronchiectasis compared to RA patients without lung 
      disease. ACPA is associated with more severe disease in both RA-ILD and 
      RA-bronchiectasis even in the absence of articular symptoms. There is no clear 
      prediction of development of articular RA with high ACPA levels in the context of 
      positive ACPA and ILD; however, in RA-bronchiectasis, patients with positive 
      antibodies can develop RA within a year after diagnosis of bronchiectasis. Though 
      the primary focus of this narrative is to highlight the role of ACPA in 
      pathogenesis and clinical practice, we also discuss the current treatment options 
      and trials in RA-ILD and RA-bronchiectasis. Currently, there are no clear 
      treatment guidelines. The treatments are now focusing on using a combination of 
      immunosuppression and antifibrotic agents. Combination treatment targets both the 
      fibrotic and inflammatory components of the disease process. Further studies are 
      needed to identify the use of ACPA as a biomarker to tailor the treatment in 
      RA-ILD and RA-bronchiectasis.
CI  - (c) 2021. The Author(s).
FAU - Khan, Tanjila
AU  - Khan T
AD  - Department of Respiratory Medicine, Royal Brompton Hospital, London, UK.
FAU - Jose, Ricardo J
AU  - Jose RJ
AD  - Department of Respiratory Medicine, Royal Brompton Hospital, London, UK.
AD  - Centre for Inflammation and Tissue Repair, UCL Respiratory, UCL, London, UK.
FAU - Renzoni, Elisabetta A
AU  - Renzoni EA
AD  - Interstitial Lung Disease Unit, Royal Brompton Hospital, Margaret Turner Warwick 
      Centre for Fibrosing Lung Disease, NHLI, Imperial College London, London, UK.
FAU - Mouyis, Maria
AU  - Mouyis M
AD  - Department of Rheumatology, Luton and Dunstable University Hospital, Luton, UK. 
      m.mouyis@nhs.net.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20210827
PL  - England
TA  - Rheumatol Ther
JT  - Rheumatology and therapy
JID - 101674543
EIN - Rheumatol Ther. 2021 Sep 27;:. PMID: 34570346
PMC - PMC8572256
OTO - NOTNLM
OT  - Anti-CCP antibody (ACPA)
OT  - Antifibrotics
OT  - Bronchiectasis
OT  - Citrullination
OT  - RA-ILD smoking
EDAT- 2021/08/28 06:00
MHDA- 2021/08/28 06:01
PMCR- 2021/08/27
CRDT- 2021/08/27 12:35
PHST- 2021/07/07 00:00 [received]
PHST- 2021/08/13 00:00 [accepted]
PHST- 2021/08/28 06:00 [pubmed]
PHST- 2021/08/28 06:01 [medline]
PHST- 2021/08/27 12:35 [entrez]
PHST- 2021/08/27 00:00 [pmc-release]
AID - 10.1007/s40744-021-00362-4 [pii]
AID - 362 [pii]
AID - 10.1007/s40744-021-00362-4 [doi]
PST - ppublish
SO  - Rheumatol Ther. 2021 Dec;8(4):1463-1475. doi: 10.1007/s40744-021-00362-4. Epub 
      2021 Aug 27.