title: Interstitial lung disease in scleroderma.
abstract: Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited. Several experimental therapies are currently in early-phase clinical trials and show promise.
authors.affiliation: Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
authors.forename: Sara R
authors.initials: SR
authors.lastname: Schoenfeld
authors.affiliation: Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA. Electronic address: fcastelino@mgh.harvard.edu.
authors.forename: Flavia V
authors.initials: FV
authors.lastname: Castelino
mesh_terms: D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D012595:Scleroderma, Systemic / Q000150:complications*
keywords: Diagnosis; Fibrosis; Interstitial lung disease; Pathogenesis; Systemic sclerosis; Treatment
references.citation: Lung. 2013 Oct;191(5):483-9
references.pmid: 23925736
references.citation: Rheumatology (Oxford). 2010 Feb;49(2):271-80
references.pmid: 19447770
references.citation: N Engl J Med. 2014 May 29;370(22):2083-92
references.pmid: 24836312
references.citation: JAMA. 2014 Jun 25;311(24):2490-8
references.pmid: 25058083
references.citation: Chest. 2014 Aug;146(2):422-36
references.pmid: 24576924
references.citation: Ann Rheum Dis. 2015 Jun;74(6):1188-94
references.pmid: 24442885
references.citation: Ann Rheum Dis. 2009 May;68(5):620-8
references.pmid: 19147617
references.citation: Semin Arthritis Rheum. 2012 Aug;42(1):42-55
references.pmid: 22464314
references.citation: Am J Respir Crit Care Med. 2010 Mar 15;181(6):604-10
references.pmid: 20007927
references.citation: Arthritis Rheum. 2010 Jul;62(7):2101-8
references.pmid: 20506355
references.citation: Semin Arthritis Rheum. 2010 Dec;40(3):241-9
references.pmid: 20494406
references.citation: J Clin Invest. 2011 Jan;121(1):277-87
references.pmid: 21135509
references.citation: Arthritis Rheum. 2011 May;63(5):1416-25
references.pmid: 21312187
references.citation: Arthritis Rheum. 2011 Jun;63(6):1707-17
references.pmid: 21370225
references.citation: Am J Respir Crit Care Med. 2011 Jul 1;184(1):92-9
references.pmid: 21474646
references.citation: Lancet. 2011 Aug 6;378(9790):498-506
references.pmid: 21777972
references.citation: Arthritis Rheum. 2012 Aug;64(8):2734-45
references.pmid: 22328118
references.citation: Inflamm Allergy Drug Targets. 2012 Aug 1;11(4):266-77
references.pmid: 22506881
references.citation: Br J Dermatol. 2012 Nov;167(5):1138-44
references.pmid: 23039171
references.citation: Chest. 2012 Sep;142(3):614-21
references.pmid: 22156609
references.citation: Eur Respir Rev. 2013 Mar 1;22(127):6-19
references.pmid: 23457159
references.citation: Transplantation. 2013 Apr 15;95(7):975-80
references.pmid: 23545509
references.citation: Am J Physiol Lung Cell Mol Physiol. 2002 Mar;282(3):L585-93
references.pmid: 11839555
references.citation: Am J Respir Crit Care Med. 2002 Jun 15;165(12):1581-6
references.pmid: 12070056
references.citation: Am J Pathol. 2003 May;162(5):1495-502
references.pmid: 12707032
references.citation: Clin Exp Rheumatol. 2003 Jul-Aug;21(4):429-36
references.pmid: 12942693
references.citation: J Rheumatol. 2004 Jun;31(6):1112-20
references.pmid: 15170923
references.citation: Clin Rheumatol. 2004 Aug;23(4):306-9
references.pmid: 15293090
references.citation: Arthritis Rheum. 1985 Jul;28(7):759-67
references.pmid: 4015723
references.citation: Radiology. 1990 Sep;176(3):755-9
references.pmid: 2389033
references.citation: Am Rev Respir Dis. 1991 Sep;144(3 Pt 1):706-13
references.pmid: 1892314
references.citation: Ann Intern Med. 1992 Mar 15;116(6):488-98
references.pmid: 1739240
references.citation: Am J Respir Cell Mol Biol. 1994 Apr;10(4):405-12
references.pmid: 7510986
references.citation: Arthritis Rheum. 1994 Sep;37(9):1283-9
references.pmid: 7945490
references.citation: Methods Mol Med. 2005;117:69-80
references.pmid: 16118446
references.citation: N Engl J Med. 2006 Jun 22;354(25):2655-66
references.pmid: 16790698
references.citation: J Rheumatol. 2006 Sep;33(9):1789-801
references.pmid: 16960939
references.citation: Arthritis Rheum. 2006 Dec;54(12):3954-61
references.pmid: 17133609
references.citation: Arthritis Rheum. 2006 Dec;54(12):3962-70
references.pmid: 17133610
references.citation: Ann Rheum Dis. 2007 Feb;66(2):169-73
references.pmid: 16868020
references.citation: J Clin Invest. 2007 Mar;117(3):557-67
references.pmid: 17332883
references.citation: Arthritis Rheum. 2007 Mar 15;57(2):318-26
references.pmid: 17330281
references.citation: Ann Rheum Dis. 2007 Jun;66(6):754-63
references.pmid: 17234652
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 Sep;293(3):L525-34
references.pmid: 17631612
references.citation: Am J Respir Crit Care Med. 2007 Nov 15;176(10):1026-34
references.pmid: 17717203
references.citation: Respir Med. 2008 Jan;102(1):150-5
references.pmid: 17822892
references.citation: Chest. 2008 Feb;133(2):455-60
references.pmid: 18071023
references.citation: Am J Respir Crit Care Med. 2008 Jun 1;177(11):1248-54
references.pmid: 18369202
references.citation: J Rheumatol. 2008 Jun;35(6):1064-72
references.pmid: 18464307
references.citation: Chest. 2008 Sep;134(3):601-5
references.pmid: 18403656
references.citation: Am J Respir Crit Care Med. 2009 Mar 1;179(5):408-13
references.pmid: 19096004
references.citation: J Clin Invest. 2009 Apr;119(4):772-87
references.pmid: 19287097
references.citation: Clin Rheumatol. 2011 Nov;30(11):1393-8
references.pmid: 21881859
references.citation: Arthritis Rheum. 2011 Nov;63(11):3540-6
references.pmid: 21769849
references.citation: Cell. 2012 Jun 8;149(6):1192-205
references.pmid: 22682243
references.citation: Curr Rheumatol Rep. 2014 Apr;16(4):411
references.pmid: 24523015