Title:Patterns of Interstitial Lung Disease in Egyptian Patients with Systemic
Sclerosis: Relation to Disease Parameters
Volume: 19
Issue: 2
Author(s): Abeer Ali Abdelati*, Akram Abd-Elmonaem Deghady, Ahmed Mohamed Abdelhady, Rim Aly Bastawy and Ahmed Shaaban
Affiliation:
- Department of Internal Medicine, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Alexandria
University, Alexandria, Egypt
Keywords:
Systemic sclerosis, interstitial, HRCT, interleukin-33, pneumonia, pleuroparenchymal fibroelastosis.
Abstract:
Background: Pulmonary involvement is the most common leading cause of morbidity
and mortality associated with systemic sclerosis. Therefore, identifying the various patterns of pulmonary
affection is crucial in the clinical management of these patients. In the current study, we
aim to investigate the patterns of interstitial lung disease (ILD) associated with SSc patients (SSc-
ILD) and their relation to serologic markers and clinical parameters.
Methods: A cross-sectional study was undertaken on thirty-four adult SSc patients who met the
2013 ACR/EULAR criteria for SSc and Forty healthy controls of matched age and sex. The patients
were subjected to history taking, clinical examination, skin assessment using the modified Rodnan
Skin Score (mRSS), chest x-ray (CXR), pulmonary function test (PFTs), and high resolution computed
tomography of the chest (HRCT). Routine laboratory tests were conducted in addition to immunologic
tests and an enzyme-linked immunosorbent assay (ELISA) to determine the IL-33 level.
Results: ILD was found in 23 SSc patients (67.6%); 20 patients had diffuse type while 3 patients
had limited type. Non-specific interstitial pneumonia (NSIP) was found in 56.5%, usual interstitial
pneumonia (UIP) was found in 21.7%, pleuroparenchymal fibroelastosis (PPFE) was found in
8.7%, and organizing pneumonia (OP) with the mixed pattern was found in 13% of SSc patients.
Additionally, the mean IL-33 level in SSc patients was 98±12.7 compared to 66.2±10.6 in the control
group (p < 0.001), with ILD patients having a significantly higher level (101.7±13.4) than those
without (90.4±6.2), and a strong positive correlation with mRSS.
Conclusion: Even in asymptomatic patients with SSc, ILD is prevalent, with NSIP being the most
common pattern. IL-33 could be considered a potential biomarker for predicting the presence of
ILD in SSc patients.
