Latest Updates about Interstitial Lung Disease

Dear Colleagues,

Interstitial lung diseases (ILDs) comprise more than 200 entities with different clinical, radiological, and pathological presentations, but they share one common feature—involvement of the lung interstitium. The prevalence of particular ILDs is low; they may be described as rare diseases according to the WHO definition. Nevertheless, morbidity and mortality of all ILDs are high, with hundreds of thousands of patients suffering and dying from them worldwide.

Some diseases, such as hypersensitivity pneumonitis or smoking-related interstitial fibrosis, have well-established etiological factors, but in most cases the etiology is unknown. Nevertheless, a genetic predisposition and aberrant molecular signaling are considered causes of many ILDs. On the other hand, some genetic mutations or polymorphisms may indicate more rapid disease progression or a worse response to treatment.

The most prevalent diagnoses are idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis, and connective-tissue disease-related ILD. The diagnostic pathway may be complicated with the need for specialized reference center involvement.

The treatment options are not very well established due to the difficulties in performing randomized clinical trials related to the small number of participants eligible.

Some of the ILDs are self-limiting or resolving after avoidance of the inciting agent. However, in around 30% of ILD patients a progressive fibrotic phenotype may develop, leading to the severe deterioration of these patients’ condition, respiratory failure, and death.

With regard to the above-mentioned problems, several new guidelines were developed in the last few years (i.e., guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis, recently updated with an additional part related to the progressive pulmonary fibrosis of other origin, and guidelines for the diagnosis and treatment of sarcoidosis and hypersensitivity pneumonitis).

Two antifibrotic drugs slowing the progression of fibrosis were developed in the last decade, but we as respiratory specialists still struggle with the problem of decreased survival and a lack of a cure for our patients.

To face the problems you meet in your everyday clinical practice and to help disseminate your research among a wide scientific audience, we invite you to share your original research articles, imaging studies, and review articles in the Special Issue of the International Journal of Molecular Sciences entitled “Latest Updates about Interstitial Lung Disease”.

This Special Issue will especially concern:

1. Molecular and genetic disorders related to the pathogenesis of ILDs;
2. New perspectives in the treatment of progressive pulmonary fibrosis (including personalized medicine);
3. Environmental exposures as the risk factors of ILD development;
4. Lung transplantation in patients with ILDs;
5. Links between ILDs and pulmonary neoplasms;
6. ILDs as a risk factor for thromboembolic disease;
7. COVID-19 as a possible trigger of ILDs.

We hope that the published articles will contribute to the scientific discussion concerning these extremely interesting and still underexplored group of diseases.

Dr. Katarzyna B. Lewandowska
Prof. Dr. Monika Szturmowicz
Guest Editors

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