title: New approaches to modulating idiopathic pulmonary fibrosis.
abstract: Until recently, idiopathic pulmonary fibrosis (IPF) has been a devastating and generally fatal disease with no effective therapeutic. New developments in understanding the biology of the disease include a growing consensus that the lesions are mainly composed of cells that originated from resident fibroblasts. New developments in therapeutics include recommendations against several treatment regimes that have been previously used. On a positive note, the orally available drug pirfenidone has been approved for use in IPF in China, Japan, India, and the European Union, but not yet in the United States. Other possibilities for managing IPF include managing gastrointestinal reflux, and limiting excessive salt intake. A variety of potential therapeutics for IPF are in clinical trials; for instance, in a Phase 1b trial, intravenous injections of a recombinant version of the normal human serum protein Serum Amyloid P (SAP, also known as PTX2) improved lung function in IPF patients.
authors: Richard H
authors: RH
authors: Gomer
mesh_terms: D000818:Animals; D003937:Diagnosis, Differential; D041981:Gastrointestinal Tract / Q000502:physiology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000276:immunology* / Q000503:physiopathology; D008168:Lung / Q000503:physiopathology; D008264:Macrophages / Q000276:immunology; D000683:Serum Amyloid P-Component / Q000276:immunology
chemical_list: D000683:Serum Amyloid P-Component
references: Am J Respir Crit Care Med. 2011 Dec 15;184(12):1330-2
references: 22174112
references: PLoS One. 2010 Mar 12;5(3):e9683
references: 20300636
references: Mucosal Immunol. 2009 Mar;2(2):103-21
references: 19129758
references: J Immunol. 2003 Nov 15;171(10):5537-46
references: 14607961
references: Nat Rev Immunol. 2011 Oct 14;11(11):723-37
references: 21997792
references: Proc Natl Acad Sci U S A. 2006 Nov 28;103(48):18284-9
references: 17114286
references: Lab Invest. 2002 Sep;82(9):1183-92
references: 12218079
references: Thorax. 2013 Jul;68(7):605-8
references: 23462155
references: Eur Respir Rev. 2013 Jun 1;22(128):158-62
references: 23728870
references: Curr Rheumatol Rep. 2006 Apr;8(2):145-50
references: 16569374
references: Immunol Res. 2010 Jul;47(1-3):185-206
references: 20087682
references: Chest. 2013 Jun;143(6):1699-1708
references: 23732584
references: Am J Respir Crit Care Med. 2010 May 15;181(10):1085-92
references: 20167844
references: Mol Med. 1994 Nov;1(1):71-81
references: 8790603
references: Respir Med. 2008 Sep;102(9):1342-8
references: 18621518
references: PLoS One. 2012;7(9):e45674
references: 23029177
references: Wound Repair Regen. 2008 Mar-Apr;16(2):266-73
references: 18318811
references: Annu Rev Pathol. 2013 Jan 24;8:241-76
references: 23092186
references: J Immunol. 2001 Jun 1;166(11):6735-41
references: 11359830
references: Am J Respir Crit Care Med. 2007 Aug 1;176(3):277-84
references: 17478620
references: J Lab Clin Med. 1995 Jun;125(6):779-85
references: 7539478
references: Expert Rev Respir Med. 2010 Feb;4(1):13-9
references: 20387288
references: J Immunol. 2001 Jul 15;167(2):647-54
references: 11441067
references: Fibrogenesis Tissue Repair. 2013 Jan 17;6(1):2
references: 23324174
references: Eur Respir J. 2006 Jan;27(1):136-42
references: 16387946
references: N Engl J Med. 2011 Sep 22;365(12):1079-87
references: 21992121
references: J Hum Hypertens. 2009 Jun;23(6):363-84
references: 19110538
references: J Thorac Dis. 2013 Feb;5(1):48-73
references: 23372951
references: Proc Natl Acad Sci U S A. 2011 Dec 27;108(52):E1475-83
references: 22123957
references: Am J Respir Crit Care Med. 2006 Apr 1;173(7):781-92
references: 16415274
references: BMJ. 2009 Nov 24;339:b4567
references: 19934192
references: J Immunol. 2003 Jul 1;171(1):380-9
references: 12817021
references: Wound Repair Regen. 2007 Jan-Feb;15(1):113-21
references: 17244327
references: Arthritis Rheum. 2003 Jan;48(1):248-54
references: 12528126
references: Ther Clin Risk Manag. 2011 Feb 08;7:39-47
references: 21339942
references: Am J Respir Crit Care Med. 2012 Jul 1;186(1):88-95
references: 22561965
references: Respirology. 2011 Jul;16(5):776-83
references: 21362103
references: Ann Intern Med. 2013 May 7;158(9):641-9
references: 23648946
references: J Allergy Clin Immunol. 2010 Oct;126(4):712-721.e7
references: 20673988
references: Eur Heart J. 2011 Dec;32(24):3073-80
references: 21705359
references: Am J Physiol Gastrointest Liver Physiol. 2011 Dec;301(6):G950-5
references: 21512158
references: Chest. 2006 Mar;129(3):794-800
references: 16537884
references: Lancet. 2011 May 21;377(9779):1760-9
references: 21571362
references: Cell Commun Signal. 2013 Apr 19;11(1):29
references: 23601247
references: Immunol Cell Biol. 2012 Apr;90(4):388-95
references: 21647172
references: Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824
references: 21471066
references: Am J Physiol. 1991 Nov;261(5 Pt 2):H1397-401
references: 1835307
references: Drug Des Devel Ther. 2012;6:261-72
references: 23055696
references: Int J Biochem Cell Biol. 2011 Jan;43(1):154-62
references: 21044893
references: PLoS One. 2011;6(7):e22659
references: 21799927
references: Fibrogenesis Tissue Repair. 2010 Jul 05;3:11
references: 20602770
references: N Engl J Med. 2012 May 24;366(21):1968-77
references: 22607134
references: PLoS Pathog. 2009 Apr;5(4):e1000371
references: 19360123
references: Pulm Pharmacol Ther. 2013 Dec;26(6):672-6
references: 23380438
references: N Engl J Med. 2010 Aug 12;363(7):620-8
references: 20484178
references: J Immunol. 2007 Sep 15;179(6):4035-44
references: 17785842
references: Clin Chim Acta. 1991 Aug 30;200(2-3):191-9
references: 1777968
references: Wound Repair Regen. 2012 May-Jun;20(3):277-83
references: 22564223
references: PLoS One. 2012;7(10):e47024
references: 23056564
references: Sci Transl Med. 2009 Nov 4;1(5):5ra13
references: 20368175
references: Am J Respir Crit Care Med. 2010 Mar 15;181(6):604-10
references: 20007927
references: Exp Hematol. 2010 Jul;38(7):548-56
references: 20303382
references: Circulation. 1998 Dec 8;98(23):2621-8
references: 9843472
references: Am J Respir Crit Care Med. 2011 Dec 15;184(12):1390-4
references: 21700909