title: Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) affects up to 15% of patients with connective tissue diseases (CTDs). Previous recommendations developed as part of larger efforts in PAH did not include detailed recommendations for patients with CTD-associated PAH. Therefore, we sought to develop recommendations for screening and early detection of CTD-associated PAH.
  
  
  METHODS
  We performed a systematic review of the literature on the screening and diagnosis of PAH in CTD. Using the RAND/University of California, Los Angeles consensus methodology, we developed case scenarios followed by 2 stages of voting. First, international experts from a variety of specialties voted anonymously on the appropriateness of each case scenario. The experts then met face-to-face to discuss and resolve discrepant votes to arrive at consensus recommendations.
  
  
  RESULTS
  The key recommendation stated that all patients with systemic sclerosis (SSc) should be screened for PAH. In addition, patients with mixed connective tissue disease or other CTDs with scleroderma features (scleroderma spectrum disorders) should be screened for PAH. It was recommended that screening pulmonary function tests (PFTs) with single-breath diffusing capacity for carbon monoxide, transthoracic echocardiogram, and measurement of N-terminal pro-brain natriuretic peptide (NT-proBNP) be performed in all patients with SSc and scleroderma spectrum disorders. In patients with SSc and scleroderma spectrum disorders, transthoracic echocardiogram and PFTs should be performed annually. The full screening panel (transthoracic echocardiogram, PFTs, and measurement of NT-proBNP) should be performed as soon as any new signs or symptoms are present.
  
  
  CONCLUSIONS
  We provide consensus-based, evidence-driven recommendations for screening and early detection of CTD-associated PAH. It is our hope that these recommendations will lead to earlier detection of CTD-associated PAH and ultimately improve patient outcomes.
authors.affiliation: University of Michigan, Ann Arbor.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.forename: Heather
authors.initials: H
authors.lastname: Gladue
authors.forename: Richard
authors.initials: R
authors.lastname: Channick
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: David
authors.initials: D
authors.lastname: Langleben
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Mathai
authors.forename: Rajeev
authors.initials: R
authors.lastname: Saggar
authors.forename: Scott
authors.initials: S
authors.lastname: Visovatti
authors.forename: Nezam
authors.initials: N
authors.lastname: Altorok
authors.forename: Whitney
authors.initials: W
authors.lastname: Townsend
authors.forename: John
authors.initials: J
authors.lastname: FitzGerald
authors.forename: Vallerie V
authors.initials: VV
authors.lastname: McLaughlin
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications*; D032921:Consensus; D042241:Early Diagnosis; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology; D011379:Prognosis
references.citation: Am J Respir Crit Care Med. 2009 Jan 15;179(2):151-7
references.pmid: 18931333
references.citation: Arthritis Care Res (Hoboken). 2012 Oct;64(10):1447-61
references.pmid: 23024029
references.citation: Arthritis Rheum. 2011 Nov;63(11):3522-30
references.pmid: 21769843
references.citation: Ann Intern Med. 2011 Oct 18;155(8):529-36
references.pmid: 22007046
references.citation: Arthritis Care Res (Hoboken). 2012 Oct;64(10):1431-46
references.pmid: 23024028
references.citation: Arthritis Rheum. 2009 Jun;60(6):1831-9
references.pmid: 19479881
references.citation: Intern Med J. 2009 Oct;39(10):682-91
references.pmid: 19220532
references.citation: Ann Rheum Dis. 2012 Aug;71(8):1335-42
references.pmid: 22307943
references.citation: Arthritis Rheum. 2008 Jan;58(1):284-91
references.pmid: 18163505
references.citation: Arthritis Rheum. 2005 Dec;52(12):3792-800
references.pmid: 16320330
references.citation: Arthritis Rheum. 2010 Dec;62(12):3741-50
references.pmid: 20722025
references.citation: Arthritis Care Res (Hoboken). 2012 May;64(5):625-39
references.pmid: 22473917
references.citation: Clin Rheumatol. 2012 Mar;31(3):557-61
references.pmid: 22105781
references.citation: Semin Respir Crit Care Med. 2009 Aug;30(4):369-75
references.pmid: 19634076
references.citation: Eur Heart J. 2010 Aug;31(15):1898-907
references.pmid: 20543192
references.citation: Arthritis Rheum. 1999 May;42(5):899-909
references.pmid: 10323445
references.citation: Public Health Nurs (1931). 1951 Jun;43(6):344
references.pmid: 14854123
references.citation: Arthritis Rheum. 2008 Jun 15;59(6):762-84
references.pmid: 18512708
references.citation: Arthritis Rheum. 2011 Sep;63(9):2790-6
references.pmid: 21547892
references.citation: Autoimmun Rev. 2013 Jan;12(3):410-5
references.pmid: 22841984
references.citation: Semin Arthritis Rheum. 2014 Feb;43(4):536-41
references.pmid: 24012044
references.citation: J Am Coll Cardiol. 2009 Apr 28;53(17):1573-619
references.pmid: 19389575
references.citation: BMJ. 2008 May 17;336(7653):1106-10
references.pmid: 18483053
references.citation: Semin Arthritis Rheum. 2011 Oct;41(2):95-105
references.pmid: 21420149
references.citation: Eur Respir Rev. 2012 Dec 1;21(126):306-12
references.pmid: 23204118
references.citation: J Am Soc Echocardiogr. 2010 Jul;23(7):685-713; quiz 786-8
references.pmid: 20620859
references.citation: J Rheumatol. 2013 Oct;40(10):1706-11
references.pmid: 23950183
references.citation: Ann Rheum Dis. 2007 Jun;66(6):754-63
references.pmid: 17234652
references.citation: Eur Respir J. 2009 Dec;34(6):1219-63
references.pmid: 19749199
references.citation: BMJ. 2004 Jun 19;328(7454):1490
references.pmid: 15205295
references.citation: Allergol Int. 2011 Dec;60(4):405-9
references.pmid: 22015567
references.citation: Ann Rheum Dis. 2014 Jul;73(7):1340-9
references.pmid: 23687283
references.citation: Arthritis Rheum. 1986 Apr;29(4):515-24
references.pmid: 3707629
references.citation: Am J Med. 1972 Feb;52(2):148-59
references.pmid: 4621694
references.citation: Arthritis Rheum. 1980 May;23(5):581-90
references.pmid: 7378088
references.citation: BMC Med Res Methodol. 2003 Nov 10;3:25
references.pmid: 14606960
references.citation: J Rheumatol. 2012 Jun;39(6):1265-74
references.pmid: 22589263
references.citation: J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S55-S66
references.pmid: 19555859
references.citation: J Rheumatol. 2010 Nov;37(11):2290-8
references.pmid: 20810505
references.citation: Rheumatology (Oxford). 2004 Apr;43(4):461-6
references.pmid: 15024134
references.citation: Int J Cardiol. 2008 Jul 21;127(3):410-2
references.pmid: 17689710
references.citation: J Nucl Med. 2007 May;48(5):680-4
references.pmid: 17475953
references.citation: Arthritis Care Res (Hoboken). 2010 Nov;62(11):1515-26
references.pmid: 20662044