Screening for connective tissue disease in pulmonary arterial hypertension

Ricardo J. Pagán, Augustine S. Lee, Christopher O. Austin, Charles Dwayne Burger

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Objectives: To evaluate the utility of anti-nuclear antibody (ANA) levels in distinguishing the cause of pulmonary arterial hypertension as idiopathic (IPAH) or connective tissue disease related (CTD-PAH). Methods: We retrospectively identified patients with IPAH or CTD-PAH seen between 2010 and 2012 at our institution. Medical records were reviewed for demographic and clinical data and laboratory values. Results: Of 115 patients identified, 65 (56%) had IPAH and 50 (44%) had CTD-PAH. The mean age was 59 years and most of the patients (76%) were women. Most patients (64%) were in World Health Organization functional class III or IV. Compared with the IPAH group, the CTD-PAH group had significantly increased B-type natriuretic peptide levels (635 vs 325 pg/mL; P = 0.02) and decreased pulmonary vascular resistance (6 vs 9 WU; P = 0.04). The median ANA level was significantly higher in the CTD-PAH group than the IPAH group (7 vs 0 U; P <0.001). The area under the receiver operating characteristic curve for a positive ANA to predict CTD-PAH was 0.91 (P <0.001). A cutoff of 5 U for predicting ANA provided an optimal specificity of 94% and a sensitivity of 70%. The resulting likelihood ratio using the same cutoff was 12 (P <0.001), or a positive predictive value of 91% with a negative predictive value of 79%. Conclusions: In this selected cohort of patients, a quantitative ANA value >5 U may be useful in distinguishing CTD-PAH from IPAH, but a lower level does not confidently exclude CTD-PAH.

Original languageEnglish (US)
Pages (from-to)666-669
Number of pages4
JournalSouthern Medical Journal
Volume107
Issue number10
DOIs
StatePublished - 2014

Fingerprint

Connective Tissue Diseases
Pulmonary Hypertension
Anti-Idiotypic Antibodies
Brain Natriuretic Peptide
Vascular Resistance
Medical Records
Demography

Keywords

  • Anti-nuclear antibody
  • Connective tissue disease
  • Pulmonary arterial hypertension

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Screening for connective tissue disease in pulmonary arterial hypertension. / Pagán, Ricardo J.; Lee, Augustine S.; Austin, Christopher O.; Burger, Charles Dwayne.

In: Southern Medical Journal, Vol. 107, No. 10, 2014, p. 666-669.

Research output: Contribution to journalArticle

Pagán, Ricardo J. ; Lee, Augustine S. ; Austin, Christopher O. ; Burger, Charles Dwayne. / Screening for connective tissue disease in pulmonary arterial hypertension. In: Southern Medical Journal. 2014 ; Vol. 107, No. 10. pp. 666-669.
@article{8dda01d198a0428eb0e48d37b0d77b60,
title = "Screening for connective tissue disease in pulmonary arterial hypertension",
abstract = "Objectives: To evaluate the utility of anti-nuclear antibody (ANA) levels in distinguishing the cause of pulmonary arterial hypertension as idiopathic (IPAH) or connective tissue disease related (CTD-PAH). Methods: We retrospectively identified patients with IPAH or CTD-PAH seen between 2010 and 2012 at our institution. Medical records were reviewed for demographic and clinical data and laboratory values. Results: Of 115 patients identified, 65 (56{\%}) had IPAH and 50 (44{\%}) had CTD-PAH. The mean age was 59 years and most of the patients (76{\%}) were women. Most patients (64{\%}) were in World Health Organization functional class III or IV. Compared with the IPAH group, the CTD-PAH group had significantly increased B-type natriuretic peptide levels (635 vs 325 pg/mL; P = 0.02) and decreased pulmonary vascular resistance (6 vs 9 WU; P = 0.04). The median ANA level was significantly higher in the CTD-PAH group than the IPAH group (7 vs 0 U; P <0.001). The area under the receiver operating characteristic curve for a positive ANA to predict CTD-PAH was 0.91 (P <0.001). A cutoff of 5 U for predicting ANA provided an optimal specificity of 94{\%} and a sensitivity of 70{\%}. The resulting likelihood ratio using the same cutoff was 12 (P <0.001), or a positive predictive value of 91{\%} with a negative predictive value of 79{\%}. Conclusions: In this selected cohort of patients, a quantitative ANA value >5 U may be useful in distinguishing CTD-PAH from IPAH, but a lower level does not confidently exclude CTD-PAH.",
keywords = "Anti-nuclear antibody, Connective tissue disease, Pulmonary arterial hypertension",
author = "Pag{\'a}n, {Ricardo J.} and Lee, {Augustine S.} and Austin, {Christopher O.} and Burger, {Charles Dwayne}",
year = "2014",
doi = "10.14423/SMJ.0000000000000175",
language = "English (US)",
volume = "107",
pages = "666--669",
journal = "Southern Medical Journal",
issn = "0038-4348",
publisher = "Lippincott Williams and Wilkins",
number = "10",

}

TY - JOUR

T1 - Screening for connective tissue disease in pulmonary arterial hypertension

AU - Pagán, Ricardo J.

AU - Lee, Augustine S.

AU - Austin, Christopher O.

AU - Burger, Charles Dwayne

PY - 2014

Y1 - 2014

N2 - Objectives: To evaluate the utility of anti-nuclear antibody (ANA) levels in distinguishing the cause of pulmonary arterial hypertension as idiopathic (IPAH) or connective tissue disease related (CTD-PAH). Methods: We retrospectively identified patients with IPAH or CTD-PAH seen between 2010 and 2012 at our institution. Medical records were reviewed for demographic and clinical data and laboratory values. Results: Of 115 patients identified, 65 (56%) had IPAH and 50 (44%) had CTD-PAH. The mean age was 59 years and most of the patients (76%) were women. Most patients (64%) were in World Health Organization functional class III or IV. Compared with the IPAH group, the CTD-PAH group had significantly increased B-type natriuretic peptide levels (635 vs 325 pg/mL; P = 0.02) and decreased pulmonary vascular resistance (6 vs 9 WU; P = 0.04). The median ANA level was significantly higher in the CTD-PAH group than the IPAH group (7 vs 0 U; P <0.001). The area under the receiver operating characteristic curve for a positive ANA to predict CTD-PAH was 0.91 (P <0.001). A cutoff of 5 U for predicting ANA provided an optimal specificity of 94% and a sensitivity of 70%. The resulting likelihood ratio using the same cutoff was 12 (P <0.001), or a positive predictive value of 91% with a negative predictive value of 79%. Conclusions: In this selected cohort of patients, a quantitative ANA value >5 U may be useful in distinguishing CTD-PAH from IPAH, but a lower level does not confidently exclude CTD-PAH.

AB - Objectives: To evaluate the utility of anti-nuclear antibody (ANA) levels in distinguishing the cause of pulmonary arterial hypertension as idiopathic (IPAH) or connective tissue disease related (CTD-PAH). Methods: We retrospectively identified patients with IPAH or CTD-PAH seen between 2010 and 2012 at our institution. Medical records were reviewed for demographic and clinical data and laboratory values. Results: Of 115 patients identified, 65 (56%) had IPAH and 50 (44%) had CTD-PAH. The mean age was 59 years and most of the patients (76%) were women. Most patients (64%) were in World Health Organization functional class III or IV. Compared with the IPAH group, the CTD-PAH group had significantly increased B-type natriuretic peptide levels (635 vs 325 pg/mL; P = 0.02) and decreased pulmonary vascular resistance (6 vs 9 WU; P = 0.04). The median ANA level was significantly higher in the CTD-PAH group than the IPAH group (7 vs 0 U; P <0.001). The area under the receiver operating characteristic curve for a positive ANA to predict CTD-PAH was 0.91 (P <0.001). A cutoff of 5 U for predicting ANA provided an optimal specificity of 94% and a sensitivity of 70%. The resulting likelihood ratio using the same cutoff was 12 (P <0.001), or a positive predictive value of 91% with a negative predictive value of 79%. Conclusions: In this selected cohort of patients, a quantitative ANA value >5 U may be useful in distinguishing CTD-PAH from IPAH, but a lower level does not confidently exclude CTD-PAH.

KW - Anti-nuclear antibody

KW - Connective tissue disease

KW - Pulmonary arterial hypertension

UR - http://www.scopus.com/inward/record.url?scp=84961388322&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84961388322&partnerID=8YFLogxK

U2 - 10.14423/SMJ.0000000000000175

DO - 10.14423/SMJ.0000000000000175

M3 - Article

VL - 107

SP - 666

EP - 669

JO - Southern Medical Journal

JF - Southern Medical Journal

SN - 0038-4348

IS - 10

ER -