The Role of Pulmonary Function Test for Pulmonary Arterial Hypertension in Patients with Connective Tissue Disease.

Objective

The study aimed to investigate the value of pulmonary function test (PFT) in evaluating and predicting pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD).

Methods

This was a prospective observational study recruiting patients diagnosed with CTD-PAH. Patients with interstitial lung disease and pulmonary hypertension induced by other causes were not eligible for enrollment. All patients were assessed for PAH every 1-3 months. A patient was considered to have clinical improvement if the grade of risk stratification declined or at least two parameters improved during follow-up, otherwise no improvement.

Results

A total of 31 patients with CTD-PAH were recruited in this study. Nearly 70% of patients had declined forced vital capacity (FVC), 60% had declined total lung capacity and maximum expiratory flow at 50% of vital capacity, and 95% had normal or mild decline in forced expiratory volume in 1 second (FEV1)/FVC. A decline in diffusing capacity of the lung for carbon monoxide (DLCO) was present in 96% of patients, and 60% were moderate to severe. Furthermore, 50% of patients had an FVC/DLCO ratio of less than 1.4. Univariate analysis showed that FEV1/FVC, DLCO, and FVC/DLCO were associated with disease prognosis. After adjusting for age as a confounding factor, multivariate logistic regression analysis revealed that DLCO was an independent predictive factor for the prognosis of CTD-PAH.

Conclusion

The pulmonary function of patients with CTD-PAH is abnormal in parameters such as lung volume, small airway, and gas exchange. PFT can reveal complex pathophysiological changes in the lungs of CTD-PAH patients and predict prognosis.