Evaluation of patients diagnosed with primary Sjogren’s syndrome with and without pulmonary involvement

Zehra Ozsoy; Tuğçe Şahin Ozdemırel; Kerem  Ensarıoğlu; Hakan  Erturk; Zeynep  Ozturk; Hasret  Yıldırım; Esma Sevil  Akkurt; Esin  Beyan; Umut  Kalyoncu; Berna Akıncı  Ozyurek

doi:10.36141/svdld.v42i3.17364

Authors

Zehra Ozsoy Division of Rheumatology, Department of Internal Medicine, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Tuğçe Şahin Ozdemırel Department of Pulmonology, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Kerem Ensarıoğlu Department of Pulmonology, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Hakan Erturk Department of Radiology, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Zeynep Ozturk Division of Rheumatology, Department of Internal Medicine, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Hasret Yıldırım Department of Pulmonology, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Esma Sevil Akkurt Department of Pulmonology, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Esin Beyan Department of Internal Medicine, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey
Umut Kalyoncu Division of Rheumatology, Department of Internal Medicine, Hacettepe University Faculty of Medicine, University of Health Sciences, Ankara, Turkey
Berna Akıncı Ozyurek Department of Pulmonology, Ankara Atatürk Sanatorium Training and Research Hospital, University of Health Sciences, Ankara, Turkey

Keywords:

Sjögren’s Syndrome, pulmonary involvement, interstitial lung disease, antifibrotic therapies

Abstract

Background and aim: Interstitial lung disease (ILD) is the most severe pulmonary complicationin Primary Sjögren’s Syndrome (pSS). We aimed to evaluate and compare the pulmonary involvement patterns, respiratory parameters, clinical, radiological, pathological, and laboratory features, disease activity scores, treatment choices, and the relationships between these findings at diagnosis in pSS patients with and without pulmonary involvement.

Methods: Patients at Ankara Ataturk Sanatorium Training and Research Hospital were included in the analysis. Patients with ILD who met the classification criteria were included as the study group. Patients who met the SS classification criteria but had no findings in the lungs were included as the control group.

Results: The median disease duration of ILD patients included in the study was 9.98 months. In pSS patients, patients with ILD had demographic (older age, male gender, more frequent smoking), symptomatology (frequent dry eyes), auto-antibody positivity (more frequent antinuclear antibodies (ANA), anti-Sjögren’s Syndrome-related antigen A (SS-A), anti-Sjögren’s Syndrome-related antigen B (SS-B)), higher disease activity and more frequent immunosuppressive use. When patients with pSS and ILD were compared according to high resolution computed tomography (HRCT) uptake pattern as nonspecific interstitial pneumonia (NSIP) (fibrotic or not) and unclassified, there were more male gender, lower forced vital capacity (FVC) values, and more frequent immunosuppressive and anti-fibrotic use in the NSIP group. When patients with ILD were classified according to gender, males had more smoking, SSB positivity, fibrotic NSIP, and lower FVC and diffusing lung capacity for carbon monoxide (DLCO) values.

Conclusion: A multidisciplinary approach involving pulmonologists, radiologists, and rheumatologists who are experts in ILD is important to increase diagnostic reliability. Pulmonary involvement in pSS is an important cause of morbidity and mortality and should be managed more carefully in male patients.

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