Are There Differences in Inflammatory and Fibrotic Pathways between IPAF, CTD-ILDs, and IIPs? A Single-Center Pilot Study

Rzepka-Wrona, Patrycja; Skoczyński, Szymon; Barczyk, Adam

doi:10.3390/ijms232315205

Cited by 5 publications

(5 citation statements)

References 33 publications

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“…Our pilot study [ 7 ] also revealed that the BALF IL-8 concentration is associated with the presence of skin teleangiectasias. IL-8 is a known proangiogenic factor.…”

Section: Discussionmentioning

confidence: 76%

“…In our pilot study [ 7 ], we revealed a greater prevalence of gastrointestinal reflux disease (GERD) in the CTD-ILD group than in the IPAF and IIP groups. Data on the impact of GERD on fibrosis pathogenesis are conflicting.…”

Section: Discussionmentioning

confidence: 99%

“…In the pilot study [ 7 ], it was also revealed that BALF levels of interleukin 8 (IL-8), CXCL1, and TGF-β1 were significantly higher in the CTD-ILD group than in the IPAF and IIP groups. We hope that the analysis of 240 subjects in this full-size study will reveal more defined differences between study groups.…”

Section: Discussionmentioning

confidence: 99%

“…Control group 2 will comprise patients with the following IIPs: IPF, nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis–associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), and lymphocytic interstitial pneumonia (LIP). Previously untreated patients with a new diagnosis of ILD were recruited for a pilot study (a separate population of patients) [ 7 ].…”

Section: Methodsmentioning

confidence: 99%

“…The recruitment process duration is estimated at 12 months. The previous recruitment of 40 patients led to a pilot study [ 7 ], which impacted further study design. The maximum number of patients included in this phase will be 80 (33.3%) in the IPAF group, 80 (33.3%) in the CTD-ILD group, and 80 (33.3%) in the IIP group.…”

Section: Methodsmentioning

confidence: 99%

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Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF): Protocol for a Multicenter Prospective Study

Rzepka-Wrona¹,

Skoczyński²,

Piotrowski³

et al. 2023

JMIR Res Protoc

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Background “Interstitial lung disease” (ILD) is a broad term encompassing diseases of different backgrounds. “Interstitial pneumonia with autoimmune features” (IPAF) is a recent term that implies the presence of autoimmunity. Objective This study aims to determine the characteristics of Polish patients with IPAF, compare them with patients with other interstitial pneumonias, and search for the prognostic and diagnostic biomarkers of IPAF in serum and bronchoalveolar lavage fluid (BALF). Methods This multicenter prospective study plans to recruit 240 participants divided into 1 study group and 2 control groups. Biological fluid samples will be collected according to Polish Respiratory Society management guidelines and stored at –80°C for further tests. Prospective 5-year observations of 60 newly diagnosed individuals are planned. The study will be divided into subsections. First, we plan to characterize Polish patients with IPAF (study group) against their peers with other ILDs (2 control groups). Control group 1 will comprise patients with idiopathic ILDs, including mainly idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Control group 2 will comprise patients with connective tissue disease–associated interstitial lung diseases, such as rheumatoid arthritis, systemic sclerosis, polymyositis, dermatomyositis, Sjögren’s syndrome, mixed connective tissue disease, and systemic lupus erythematosus. Radiological and functional parameters will be analyzed. Patients will be compared in terms of high-resolution computed tomography results, the 6-minute walking test performance, and pulmonary function test parameters. The diagnosis of IPAF will be reassessed on a regular basis through multidisciplinary discussion in order to determine its clinical stability. In the laboratory arm, inflammation and fibrosis pathways will be assessed. Cytokine levels (interleukin 8, transforming growth factor beta 1, chemokine C-C motif ligand [CXCL]18, CXCL1, surfactant protein [SP]-A, SP-D, Krebs von den Lungen-6 protein, and chitinase 1) will be measured in serum and BALF. A comparative analysis of serum and BALF cytokine levels will be performed in order to establish potential differences between systemic and local inflammatory pathways. In the quality of life (QoL) arm of the study, dyspnea and cough and their impact on various aspects of the QoL will be assessed. Depression and anxiety will be measured with the Hospital Anxiety and Depression Modified Scale and the 9-item Patient Health Questionnaire, and potential correlations with symptom prevalence will be assessed. Results This study will start recruiting patients to phase 1 in October 2023. The final results will be available in 2028. We plan to publish preliminary results after 2-3 years from the start of phase 1. Conclusions This study will be a step toward a better understanding of IPAF etiopathogenesis and outcomes. International Registered Report Identifier (IRRID) PRR1-10.2196/44802

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“…Our pilot study [ 7 ] also revealed that the BALF IL-8 concentration is associated with the presence of skin teleangiectasias. IL-8 is a known proangiogenic factor.…”

Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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