JOURNAL ARTICLE

Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension

Amber Young, Vivek Nagaraja, Mark Basilious, Mirette Habib, Whitney Townsend, Heather Gladue, David Badesch, J Simon R Gibbs, Deepa Gopalan, Alessandra Manes, Ronald Oudiz, Toru Satoh, Adam Torbicki, Fernando Torres, Vallerie McLaughlin, Dinesh Khanna
Seminars in Arthritis and Rheumatism 2019, 48 (6): 1059-1067
30415942

OBJECTIVE: Pulmonary arterial hypertension (PAH) has high morbidity and mortality in connective tissue diseases (CTDs), especially systemic sclerosis (SSc). In this systematic review, we provide an update on screening measures for early detection of PAH in CTD.

METHODS: Manuscripts published between July 2012 and October 2017, which incorporated screening measures to identify patients with PAH by right heart catheterization, were identified. Risk of bias was assessed using the QUADAS-2 tool.

RESULTS: The systematic review resulted in 1514 unique citations and 22 manuscripts were included for final review; the majority of manuscripts had a lower risk of bias based on the QUADAS-2 tool. There were 16 SSc cohort studies and 6 case-control studies (SSc 4, SLE 2). Four SSc cohort studies evaluated transthoracic echocardiography (TTE) only. Eight SSc cohort studies evaluated composite measures including ASIG, DETECT, and a combination of tricuspid regurgitation velocity (TRV) and PFT variables. DETECT and ASIG had greater sensitivity and negative predictive value (NPV) compared to the 2009 ESC/ERS guidelines in different cohorts. The addition of PFT variables, such as DLCO or FVC/ DLCO ratio, to TRV, resulted in greater sensitivity and NPV compared to TRV alone.

CONCLUSION: Current screening for PAH in CTDs is centered on SSc. Data continues to support the use of TTE and provides additional evidence for use of composite measures.

Full Text Links

Find Full Text Links for this Article

Discussion

You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read
30415942
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"