Walking the path of treatable traits in interstitial lung diseases

Amati, Francesco; Spagnolo, Paolo; Ryerson, Christopher J.; Oldham, Justin M.; Gramegna, Andrea; Stainer, Anna; Mantero, Marco; Sverzellati, Nicola; Lacedonia, Donato; Richeldi, Luca; Blasi, Francesco; Aliberti, Stefano

doi:10.1186/s12931-023-02554-8

Respiratory Research

Table 3 Molecular biomarkers identified in ILDs that are associated with relevant outcomes

From: Walking the path of treatable traits in interstitial lung diseases

Biomarker	Matrix	Disease*	Field of action	Prognostic relevance	Potential treatment
Short telomere length [34, 30, 97,98,99,100,101]	Peripheral blood leucocytes	IPF HP Unclassifiable ILD IPAF CTD-ILD	Dysfunctional alveolar repair	Higher post-transplant morbidity Significantly increased risk of harm in patients receiving immunomodulatory treatment	Placenta derived mesenchymal stromal cells
TOLLIP gene variant [31, 102]	Peripheral blood leucocytes	IPF	Immune dysregulation	Lung function decline Mortality	NAC
MUC5B promoter polymorphism [97,98,99, 103]	Peripheral blood leucocytes	IPF	Dysfunctional alveolar repair	Mortality	Unknown
KL-6 [104,105,106,107,108,109,110,111,112,113]	Serum BAL	CTD-ILD NSIP PAP CHP IPF	Dysfunctional alveolar repair	Severity of disease Risk of progression Risk of exacerbation Radiological scores	Unknown
Surfactant protein D [104,105,106,107,108,109, 112, 113]	Serum	IPF	Dysfunctional alveolar repair	Risk of progression	Unknown
MMP-7 [105, 106, 110, 114]	Serum	IPF CHP	Extracellular matrix turnover and remodeling	Lung function decline Mortality	Unknown
YKL-40 [104, 115, 119, 120]	Serum BAL	IPF CHP CTD-ILD Sarcoidosis	Extracellular matrix turnover and remodeling	Risk of progression Risk of exacerbation Mortality	Unknown
CCL-18 [104, 111, 116]	Serum	IPF	Immune dysregulation	Lung function decline Mortality	Unknown
IL-6 [117, 118]	Serum	CTD-ILD	Immune dysregulation	Lung function decline Mortality	Tocilizumab
PRO-C3 and PRO-C6 [121, 122]	Serum	IPF	Extracellular matrix turnover and remodeling	Risk of progression	Unknown
CRPM [123]	Serum	IPF	Extracellular matrix turnover and remodeling	Lung function decline	Unknown
Periostin [124]	Serum	IPF CHP	Extracellular matrix turnover and remodeling	Lung function decline Mortality Risk of exacerbation	Unknown
VCAM-1 [125, 126]	Serum	CTD-ILD IPF Unclassifiable ILD CHP	Immune dysregulation	Lung function decline Mortality	Unknown
CXCL 13 [126, 127]	Serum	CTD-ILD CHP IPF Unclassifiable ILD	Immune dysregulation	Lung function decline Mortality	Unknown

IPF idiopathic pulmonary fibrosis, HP hypersensitivity pneumonitis, IPAF interstitial pneumonia with autoimmune features, CTD-ILD connective tissue disease-interstitial lung disease, TOLLIP toll interacting protein, NAC N-acetyl cysteine, MUC5B Mucin 5B, KL-6 Krebs von den Lungen 6, BAL bronchoalveolar lavage, NSIP non-specific interstitial pneumonia, PAP pulmonary alveolar proteinosis, MMP-7 matrix metallopreinase 7, YKL-40 chitinase-3-like protein 1, CCL-18 C–C Motif Chemokine Ligand 18, IL-6 Interlukin-6, CRPM serum matrix metalloproteinase-degraded C-reactive protein, VCAM-1 vascular cell adhesion protein 1, CXCL 13 C-X-C motif chemokine 13
^*Disease(s) in which the biomarker has been identified

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ISSN: 1465-993X

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