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

Overview

Journal Dis Markers

Publisher Wiley

Specialty Biochemistry

Date 2022 Oct 10

PMID 36212174

Authors

Jiangbiao Xiong

Jianbin Li

Yiping Huang

Fan Yang

Rui Wu

Affiliations

Soon will be listed here.

Abstract

: 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). : 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. : 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. : 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.

Citing Articles

Multimodal Screening for Pulmonary Arterial Hypertension in Systemic Scleroderma: Current Methods and Future Directions.

Dragoi I, Rezus C, Burlui A, Bratoiu I, Rezus E Medicina (Kaunas). 2025; 61(1).

PMID: 39859001 PMC: 11766816. DOI: 10.3390/medicina61010019.

References

Bournia V, Kallianos A, Panopoulos S, Gialafos E, Velentza L, Vlachoyiannopoulos P . Cardiopulmonary exercise testing and prognosis in patients with systemic sclerosis without baseline pulmonary hypertension: a prospective cohort study. Rheumatol Int. 2021; 42(2):303-309. DOI: 10.1007/s00296-021-04937-w. View

Low A, George S, Howard L, Bell N, Millar A, Tulloh R . Lung Function, Inflammation, and Endothelin-1 in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension. J Am Heart Assoc. 2018; 7(4). PMC: 5850183. DOI: 10.1161/JAHA.117.007249. View

Goh N, Veeraraghavan S, Desai S, Cramer D, Hansell D, Denton C . Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum. 2007; 56(6):2005-12. DOI: 10.1002/art.22696. View

Jiang Y, Turk M, Pope J . Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). Autoimmun Rev. 2020; 19(9):102602. DOI: 10.1016/j.autrev.2020.102602. View

Soon E, Holmes A, Treacy C, Doughty N, Southgate L, Machado R . Elevated levels of inflammatory cytokines predict survival in idiopathic and familial pulmonary arterial hypertension. Circulation. 2010; 122(9):920-7. DOI: 10.1161/CIRCULATIONAHA.109.933762. View

Diamanti E, Karava V, Yerly P, Aubert J . Carbon Monoxide Diffusion Capacity as a Severity Marker in Pulmonary Hypertension. J Clin Med. 2022; 11(1). PMC: 8745155. DOI: 10.3390/jcm11010132. View

Sobiecka M, Lewandowska K, Kober J, Franczuk M, Skoczylas A, Tomkowski W . Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?. Lung. 2020; 198(3):547-554. PMC: 7242254. DOI: 10.1007/s00408-020-00346-1. View

Gaine S, Chin K, Coghlan G, Channick R, Di Scala L, Galie N . Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. Eur Respir J. 2017; 50(2). PMC: 5593379. DOI: 10.1183/13993003.02493-2016. View

Galie N, Humbert M, Vachiery J, Gibbs S, Lang I, Torbicki A . 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS):.... Eur Respir J. 2015; 46(4):903-75. DOI: 10.1183/13993003.01032-2015. View

10.

Farber H, Miller D, Poms A, Badesch D, Frost A, Muros-Le Rouzic E . Five-Year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015; 148(4):1043-54. DOI: 10.1378/chest.15-0300. View

11.

Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J . Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005; 52(12):3792-800. DOI: 10.1002/art.21433. View

12.

Sun X, Hansen J, Oudiz R, Wasserman K . Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol. 2003; 41(6):1028-35. DOI: 10.1016/s0735-1097(02)02964-9. View

13.

Cogswell R, Pritzker M, De Marco T . Performance of the REVEAL pulmonary arterial hypertension prediction model using non-invasive and routinely measured parameters. J Heart Lung Transplant. 2014; 33(4):382-7. DOI: 10.1016/j.healun.2013.12.015. View

14.

Hoeper M, Bogaard H, Condliffe R, Frantz R, Khanna D, Kurzyna M . Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl):D42-50. DOI: 10.1016/j.jacc.2013.10.032. View

15.

Meyer F, Ewert R, Hoeper M, Olschewski H, Behr J, Winkler J . Peripheral airway obstruction in primary pulmonary hypertension. Thorax. 2002; 57(6):473-6. PMC: 1746348. DOI: 10.1136/thorax.57.6.473. View

16.

Rhee R, Gabler N, Sangani S, Praestgaard A, Merkel P, Kawut S . Comparison of Treatment Response in Idiopathic and Connective Tissue Disease-associated Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2015; 192(9):1111-7. PMC: 4642205. DOI: 10.1164/rccm.201507-1456OC. View

17.

Tuder R . Pathology of pulmonary arterial hypertension. Semin Respir Crit Care Med. 2009; 30(4):376-85. DOI: 10.1055/s-0029-1233307. View

18.

DAlto M, Dimopoulos K, Coghlan J, Kovacs G, Rosenkranz S, Naeije R . Right Heart Catheterization for the Diagnosis of Pulmonary Hypertension: Controversies and Practical Issues. Heart Fail Clin. 2018; 14(3):467-477. DOI: 10.1016/j.hfc.2018.03.011. View

19.

Badesch D, Raskob G, Elliott C, Krichman A, Farber H, Frost A . Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2009; 137(2):376-87. DOI: 10.1378/chest.09-1140. View

20.

Dorfmuller P, Humbert M . Progress in pulmonary arterial hypertension pathology: relighting a torch inside the tunnel. Am J Respir Crit Care Med. 2012; 186(3):210-2. DOI: 10.1164/rccm.201206-1049ED. View