This research topic collection entitled “Pulmonary Fibrosis: one manifestation, various
diseases
”, involving authors from different countries, confirms that this disease is a hot
topic (Confalonieri P et al.,2022, Orlandi M et al., 2022). There are over 200 different
types of pulmonary fibrosis (PF), the most common is the idiopathic pulmonary fbrosis
(IPF), called idiopathic because it has no known cause. Another rare form is familial
PF, for which several studies reported correlation with few genes. An important group
of PF are due to other diseases, for example, autoimmune diseases such as rheumatoid
arthritis, systemic sclerosis or Sjogren’s syndrome (Ruaro et al., 2022, Trombetta
AC et al., 2017, Bernero Eet al., 2013). PF could correlate to viral infections (e.g.
COVID-19), gastroesophageal reflux disease (GERD) (Baratella E et al, 2021, Ruaro
et al., 2018), and the exposure to various materials (including naturally occurring
such as bird or animal droppings, and occupational such as asbestos or silica). Furthermore,
smoking, radiation treatments, and certain drugs can increase risk of developing PF.
In the first article (Saketkoo et al.) of the collection, the authors evaluate the
use of International Classification of Functioning, Disability, and Health (ICF) approved
by World Health Organization (WHO) in patients affected by interstitial lung diseases
(ILD). The results of the study supported the use of ICF in ILD, as ICF may help clinicians
to collect data regarding the clinical status of their ILD patients.
The second article (Ma et al.) of the collection is an interesting and comprehensive
review. The authors underlined the molecular mechanisms and pathogenic factors of
IPF, which would be helpful in the diagnosis, development of new drugs and the improvement
of disease prognosis. In particular, the researchers underlined the novelties regarding
multiple cell types, gene mutations, epigenetic and environmental factors.
The most important message reported in the third paper (Zhou et al.) is that the assessments
by high-resolution computed tomography (HRCT) pattern and scores before transbronchial
cryobiopsy (TBCB) were helpful for bronchoscopists to make a better patient selection
and procedure planning. The authors also reported that the multivariate analysis supported
radiological probable interstitial pneumonia (UIP) pattern as an independent risk
factor for moderate bleeding.
The fourth article (Zhang et al.) is a case report. The authors performed a transbronchial
cryobiopsy (TBCB) assisted by extracorporeal membrane oxygenation (ECMO) in a critical
case of acute respiratory failure related to an organizing pneumonia (OP) pattern.
In conclusion, the paper supported that when oxygenation cannot be maintained after
endotracheal intubation and surgical lung biopsy is not feasible, TBCB supported by
ECMO may be a good choice to obtain lung tissue for histopathological diagnosis in
patients with acute lung injury of unknown etiology.
The fifth manuscript (Zhou et al.) is an interesting case report that evaluate the
treatment by pirfenidone of PF secondary to ARDS-COVID-19. Over 96 weeks after pirfenidone,
the score of the mMRC dyspnea scale, the 6 min walking test distance, total lung capacity,
diffusion capacity for carbon monoxide and chest CT improved. In conclusion, this
case demonstrated that pirfenidone might be a potential treatment option for the post-COVID-19
pulmonary fibrosis.
The sixth article (Wang et al.) is a retrospective study that evaluate 579 patients
with fibrosing ILD, of which 227 (39%) met the criteria for progression. The authors
observed that clubbing of fingers and a HRCT-documented UIP-like fibrotic pattern
were more frequently associated with the progressive fibrosing.
The mortality was worse in patients with PF with hypoxemia, in those with baseline
diffusion capacity of the lung for carbon monoxide (DLCO)% predicted <50%, or in those
with UIP-like fibrotic pattern.
In the seventh paper (Ma et al.) the researchers provides an overview of different
cytokines and growth factors involved in IPF.
The authors of the eighth article (Min et al.) demonstrated that lungs from mice with
bleomycin (BLM)-induced PF were characterized by decreased expression of TNF receptor-associated
factor 6 (TRAF6) in lung fibroblasts. Furthermore, the results indicate that reduced
TRAF6 expression in fibroblasts is essential for the progression of PF, and therefore,
genetically increasing TRAF6 expression or disrupting tribbles pseudokinase 3 (TRIB3)-TRAF6
interaction could be potential therapeutic strategies for fibroproliferative lung
diseases.
In the ninth article (Xu et al.) the authors used human embryonic lung fibroblasts
(HELFs) treated with different concentrations of vincristine (VCR) to study the molecular
mechanism of VCR-induced PF and the possible involvement of the mitogen-activated
protein kinase (MAPK) signaling pathway. In the conclusions, the researchers reported
that VCR could promote the differentiation of fibroblasts into myofibroblasts by regulating
the MAPK signal pathway.
In the penultimate article of the collection, the authors (Tanner et al.) used a series
of in vitro and in vivo models to identify the therapeutic potential of bisphosphonate
zoledronic acid (ZA) in the treatment of idiopathic pulmonary fibrosis (IPF). Furthermore,
farnesyl diphosphate synthase (FDPS) was used as a potential antifibrotic target using
a bleomycin mouse model. The results of the study reported that in vitro administration
of ZA reduced myofibroblast transition and blocked NF-κB signaling in macrophages
leading to impaired immune cell recruitment in a transwell assay. FDPS-targeting siRNA
administration significantly attenuated profibrotic cytokine production and lung damage.
In addition, ZA treatment of mice with bleomycin-induced lung damage displayed decreased
cytokine levels in the BALF, plasma, and lung tissue, resulting in less histologically
visible fibrotic scarring. Additionally, ZA polarized macrophages towards a less profibrotic
phenotype contributing to decreased IPF pathogenesis.
The last research (Yu et al.) proved that catalpol (CAT) might work through Ang Ⅱ/AT1/TGF-β/Smads
pathway to improve lung pathological changes as well as suppress epithelial mesenchymal
transition (EMT) in mice with PF. CAT may serve as a novel therapeutic candidate for
the simultaneous blockade of Ang II and TGF-β pathway to attenuate PF.
In conclusion, this special issue pays particular attention to recently progress made
on use of innovative tests and treatments, which is expected to provide new insights
into research.