J Rheum Dis.  2018 Jul;25(3):179-187. 10.4078/jrd.2018.25.3.179.

Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

• Jung JY
• Lee CH
• Kim HA
• Choi ST
• Lee JH
• Yoon BY
• Kang DR
• Suh CH

• KMID: 2415606
• DOI: http://doi.org/10.4078/jrd.2018.25.3.179

Abstract

OBJECTIVE
Pulmonary hypertension (PH) develops frequently in connective tissue diseases (CTD) and is an important prognostic factor. The aim of this study was to assess the prevalence of PH in patients with CTD by non-invasive echocardiography and analyze the potential biomarkers for helping to detect PH.
METHODS
All Korean patients with CTD who had dyspnea on exertion or interstitial lung disease (ILD) were screened for PH with echocardiography and clinical data were collected from four hospitals.
RESULTS
Among 196 patients with CTD, 108 (55.1%) had ILD and 21 had PH defined as >40 mmHg. Of the 21 patients with PH, 10, 4, and 3 patients had systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease, respectively. There was no difference in the incidence of PH according to the presence of ILD; 12 patients (11.1%) with ILD had PH and 9 patients (10.2%) without ILD had PH. The results of the pulmonary function test, total cholesterol, red cell volume distribution width, alkaline phosphatase, and the New York Heart Association (NYHA) functional class III or IV differed significantly according to the presence of PH. In multiple regression analysis, NYHA functional class III or IV (odd ratio [OR]=7.3, p=0.009) and forced vital capacity (OR=0.97, p=0.043) were independent predictive factors of PH.
CONCLUSION
PH is not associated with the presence of ILD in Korean patients with CTD. On the other hand, NYHA functional class III or IV and decreased forced vital capacity indicate the presence of PH in connective tissue disease.

Keyword

Connective tissue disease; Pulmonary hypertension; Echocardiography; Interstitial lung disease; Risk factors

MeSH Terms

Alkaline Phosphatase
Biomarkers
Cell Size
Cholesterol
Connective Tissue Diseases*
Connective Tissue*
Dyspnea
Echocardiography
Hand
Heart*
Humans
Hydrogen-Ion Concentration
Hypertension, Pulmonary*
Incidence
Lung Diseases, Interstitial*
Lupus Erythematosus, Systemic
Mixed Connective Tissue Disease
Prevalence
Respiratory Function Tests
Risk Factors
Scleroderma, Systemic
Vital Capacity*
Alkaline Phosphatase
Biomarkers
Cholesterol

Figure

• 

  Figure 1. Receiver operating characteristic to predict pulmonary hypertension by echocardiography in connective tissue disease. The area under curve (AUC) of receiver operating characteristic by final model with New York Heart Association functional class III or IV and forced vital capacity was 0.767.

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