JOPIC
Journal of Pulmonology and Intensive Care

The JoPIC is an independent-unbiased, peer-reviewed, and open-access journal of current national and international issues and reviews for original clinical and experimental research, interesting case reports, surgical techniques, differential diagnoses, editorial opinions, letters to the editor, and educational papers in pulmonology, thoracic surgery, occupational diseases, allergology, and intensive care medicine.

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Case Report
Systemic sclerosis-associated interstitial lung disease complicated by precapillary pulmonary hypertension: a real-world case
Abstract
Introduction: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by widespread vasculopathy and progressive fibrosis. Pulmonary involvement, particularly interstitial lung disease (ILD) and pulmonary hypertension (PH), is among the leading causes of morbidity and mortality in these patients. Herein, we present a case of precapillary pulmonary hypertension that developed following interstitial lung disease (SSc-ILD) in a patient with systemic sclerosis. Case Presentation: A 60-year-old woman diagnosed with systemic sclerosis in 2019 based on positive antinuclear antibody (ANA) and anti-Scl-70 antibody findings was referred to our outpatient clinic with progressively worsening exertional dyspnea. Initial evaluation revealed a marked reduction in diffusing capacity for carbon monoxide (DLCO, 24% predicted) and a cellular nonspecific interstitial pneumonia (NSIP) pattern on high-resolution computed tomography (HRCT). Over the following years, the NSIP pattern progressed from a cellular to a fibrotic phenotype, accompanied by significant clinical deterioration by 2022. As the disease progressed to the fibrotic stage and the patient was diagnosed with progressive pulmonary fibrosis, nintedanib therapy was initiated. Right heart catheterization revealed a mean pulmonary artery pressure of 39 mmHg, confirming precapillary pulmonary hypertension and leading to a diagnosis of Group 1 pulmonary arterial hypertension (PAH). Macitentan was initiated for PAH, while nintedanib, administered for ILD, had to be discontinued because of gastrointestinal intolerance. Despite partial improvement in pulmonary artery pressures, pulmonary function continued to deteriorate, with DLCO declining to 13% predicted. The patient died of cardiopulmonary arrest in 2024. Conclusion: The coexistence of SSc-ILD and pulmonary hypertension represents a severe clinical phenotype associated with a poor prognosis. A progressive decline in DLCO may serve as a sensitive functional marker of the development of pulmonary vascular disease, while right heart catheterization remains the gold standard for definitive diagnosis. Early recognition and individualized management are crucial for optimizing outcomes in these complex patients.

Keywords : Scleroderma; systemic, lung diseases; interstitial, hypertension; pulmonary, cardiac catheterization, pulmonary diffusing capacity

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Informed Consent

Written informed consent was obtained from the patient included in this report. Signed consent forms are retained by the author and are available upon request.

Author Contributions

The author is solely responsible for the entirety of conception, execution, analysis, and writing of the manuscript.

Conflict of Interest

The author declares no conflicts of interest.

Financial Disclosure

This case report did not receive any financial support.


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Article available in :
Volume 4, Issue 4, 2026
Page : 123-126
https://doi.org/10.51271/JOPIC-0092
Article Information
Received : 15 Haz 2026
Accepted : 28 Eyl 2026
Published : 28 Eki 2026
Corresponding Author :

Feyzullah Esat Yazar: Department of Pulmonary Diseases, Faculty of Medicine, Kırıkkale University, Kırıkkale, Turkiye

[email protected]
Citation : Yazar FE. Systemic sclerosis-associated interstitial lung disease complicated by precapillary pulmonary hypertension: a real-world case. J Pulmonol Intens Care. 2026;4(4):123-126. doi:10.51271/JOPIC-0092

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