Jakob Höppner1, Maximilian Wollsching-Strobel1, Falk Schumacher2, Wolfram Windisch1, Melanie Berger1

1(Department of Pneumology, Cologne Merheim Hospital, Kliniken der Stadt Köln gGmbh, Faculty of Health/School of Medicine, Witten/Herdecke University, Cologne, Germany
2Department of Rheumatology, Krankenhaus Porz Am Rhein, Cologne, Germany

Keywords: Bronchoalveolar lavage, connective tissue disease, high-resolution computer tomography, interstitial lung disease, progressive pulmonary fibrosis, pulmonary function test.


Objectives: The aim of this study was to identify differences and similarities between connective tissue disease (CTD) patients with and without progressive pulmonary fibrosis (PPF) by applying the new guidelines.

Patients and methods: Patient characteristics and disease courses from medical records of 50 CTD-associated Interstitial lung disease (ILD) patients (33 females, 17 males; mean age: 60.1±12.9 years) were longitudinally studied between January 2018 and May 2022. Respiratory involvement in CTD patients was described, and differences in CTD patients who developed PPF compared to those who did not were identified by the 2022 ATS (American Thoracic Society)/ERS (European Respiratory Society)/JRS (Japanese Respiratory Society)/ALAT (Asociación Latinoamericana de Thórax) Guidelines on Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis in Adults.

Results: In the majority (74%) of patients, CTD was diagnosed before ILD onset. Nonspecific interstitial pneumonia was the most common high resolution computer tomography pattern, followed by the usual interstitial pneumonia pattern. On pulmonary function test, 38% had a restrictive pattern at baseline. Patients without PPF tended to have worse lung function at baseline and increased macrophage count in bronchoalveolar lavage than patients with PPF.

Conclusion: In patients without PPF, disease progression may be missed, resulting in inadequate management. Interdisciplinary management of patients with CTD with the participation of pulmonologists and precise lung function diagnostics is recommended.

Citation: Höppner J, Wollsching-Strobel M, Schumacher F, Windisch W, Berger M. Progressive pulmonary fibrosis in patients with connective tissue disease-associated interstitial lung disease: An explorative study. Arch Rheumatol 2024;39(1):46-51. doi: 10.46497/ ArchRheumatol.2024.10105.

Ethics Committee Approval

The study protocol was approved by the Witten/Herdecke University Ethics Committee (date: 05.10.2022, no: S-180/2022). The study was conducted in accordance with the principles of the Declaration of Helsinki.

Author Contributions

Study design: J.H., M.W.S., W.W., M.B.; Data collection: J.H., M.W.S., M.B.; Data analysis: J.H., M.W.S., M.B.; Data interpretation: J.H., M.W.S., F.S., E.S., W.W., M.B.; Drafting manuscript: J.H., M.W.S., M.B.; Revising manuscript content: E.S., F.S., W.W.; All authors reviewed the results and approved the final version of the manuscript.

Conflict of Interest

The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Financial Disclosure

The authors received no financial support for the research and/or authorship of this article.

Data Sharing Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.