Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016 Sep;95(39):e4827.
doi: 10.1097/MD.0000000000004827.

Undiagnosed connective tissue diseases: High prevalence in pulmonary arterial hypertension patients

Lorenzo Cavagna  1 , Veronica Codullo, Stefano Ghio, Carlo Alberto Scirè, Eleonora Guzzafame, Laura Scelsi, Silvia Rossi, Carlomaurizio Montecucco, Roberto Caporali
Affiliations

Undiagnosed connective tissue diseases: High prevalence in pulmonary arterial hypertension patients

Lorenzo Cavagna et al. Medicine (Baltimore). 2016 Sep.

Abstract

Among different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) have distinct hemodynamic and prognostic features; a correct etiologic diagnosis is thus mandatory.To estimate frequency and prognosis of previously undiagnosed CTDs in a suspect idiopathic (i) PAH cohort.Consecutive patients with PAH confirmed by right heart catheterization referred at the Cardiology Division of our Hospital without a previous rheumatological assessment or the occurrence of other conditions explaining PAH were checked for CTD by a clinical, laboratory, and instrumental evaluation. Survival in each group has also been analyzed.In our study 17 of 49 patients were classified as CTD-PAH, corresponding to a prevalence (95% CI) of 34.7% (21.7-49.6%). ANA positivity had 94% (71.3-99.9%) sensitivity and 78.1% (60-90.7%) specificity for a diagnosis of CTD-PAH; Raynaud phenomenon (RP) showed 83.3% (51.6-97.9%) sensitivity and 100% (90.5-100%) specificity for the diagnosis of Systemic Sclerosis (SSc)-PAH. At diagnosis, SSc patients were older and had a lower creatinine clearance compared with iPAH and other CTD-PAH. After a median follow-up of 44 (2-132) months, 18 of 49 (36.7%) patients died: 31.2% in the iPAH group, 20% in the CTD-, and 58.3% in the SSc-PAH group. Mortality was significantly higher in SSc-PAH (HR 3.32, 1.11-9.95, P <0.05) versus iPAH.We show a high prevalence of undiagnosed CTDs in patients with iPAH without a previous rheumatological assessment. All patients with RP were diagnosed with SSc. Our data stress the importance of a rheumatological assessment in PAH, especially because of the unfavorable prognostic impact of an associated SSc.

PubMed Disclaimer

Conflict of interest statement

The authors have no conflicts of interest to disclose.

Figures

Figure 1
Figure 1
Kaplan–Meier survival curves of the different PAH diagnostic groups. ∗P <0.05.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Simonneau G, Robbins I, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54:S43–S54. - PubMed
    1. Cottin V. Pulmonary arterial hypertension in connective tissue disease. Rev Mal Respir 2006; 23:13S61–13S72. - PubMed
    1. Avouac J, Airò P, Meune C, et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 2010; 37:2290–2298. - PubMed
    1. Cavagna L, Prisco E, Montecucco C, et al. Pulmonary arterial hypertension in antisynthetase syndrome: Comment on the article by Chatterjee and Farver (p 425–429). Arthritis Care Res (Hoboken) 2011; 63:633–634. - PubMed
    1. Ruiz-Cano MJ, Escribano P, Alonso R, et al. Comparison of baseline characteristics and survival between patients with idiopathic and connective tissue disease-related pulmonary arterial hypertension. J Heart Lung Transplant 2009; 28:621–627. - PubMed

Publication types

MeSH terms