Parameter | Author | Study organ | Number of patents | Type of trial | Study drug | Mode of action | Outcome measures |
---|---|---|---|---|---|---|---|
Growth factors | Noble[95] | Lung | 779 | RCT, phase III | Pirfenidone | Inhibition of TGF-β | Increased FVC in IPF |
Trachtman[98] | Kidney | 16 | Open-label, phase I | Fresolimumab | Antibody targeting all isoforms of TGF-β | Safety, pharmacokinetics | |
Ferguson[111] | Skin | 223 | RCT, phase I/II | Avotermin | Antibody targeting TGF-β3 | Acceleration and permanent improvement in dermal scaring | |
Dillingh[114] | Lung | 29 | RCT, phase I | rhSAP | Substitution of SAP | Reduction in SAP levels and circulating fibrocytes in healthy control and IPF patients | |
Oxidative stress | Raghu[158] | Lung | 88 | RCT, phase II | Etanercept | Blockade of TNF | Physiological and functional decrease in disease progression in IPF |
Corren[159] | Lung | 219 | RCT, phase II | Lebrikizumab | Antibody targeting IL-13 | Improved lung function in asthmatic patients | |
Horton[160] | Lung | 23 | RCT, phase III | Thalidomide | Anti-angiogenic and anti-inflammatory | Improvement of cough and respiratory quality of life in IPF | |
Intracellular enzymes and receptors | Richeldi[162] | Lung | 432 | RCT, phase II | BIBF 1120 | Tyrosine kinase inhibitor | Tendency towards reduced decline of lung function in IPF |
Daniels[108] | Lung | 119 | RCT, phase II/III | Imatinib | Tyrosine kinase inhibitor | No effect on survival and lung function | |
ECM and other | Couluris[140] | Lung | 20 | Uncontrolled, interventional study, phase II | Losartan | AT1 antagonist | Stabilization of lung function in IPF |
el-Agroudy[142] | Kidney | 162 | RCT, phase II | Losartan | AT1 antagonist | Decreased TGF-β1 plasma levels and proteinuria in renal interstitial fibrosis | |
Kuhn[182] | Skin | 10 | Prospective, open-label, phase II | Bosentan | Endothelin receptor antagonist | Reduced skin thickening in systemic sclerosis | |
Diez[141] | Heart | 34 | Uncontrolled, phase II | Losartan | AT1 antagonist | Decreased myocardial collagen content and left ventricular chamber stiffness in hypertensive patients | |
De[143] | Liver | 39 | RCT, phase II | Losartan | AT1 antagonist | Reduction of portal pressure in patients with liver cirrhosis | |
King[183] | Lung | 616 | RCT | Bosentan | Endothelin receptor antagonist | Improvement of FVC and diffusing capacity in IPF |