Pirfenidone and Nintedanib are specific drugs used against idiopathic pulmonary fibrosis (IPF) that showed efficacy in non-IPF fibrosing interstitial lung diseases (ILD). Both drugs have side effects that affect patients in different ways and have different levels of severity, making treatment even more challenging for patients and clinicians. The present review aims to assess the effectiveness and potential complications of Pirfenidone and Nintedanib treatment regimens across various ILD diseases. A detailed search was performed in relevant articles published between 2018 and 2023 listed in PubMed, UpToDate, Google Scholar, and ResearchGate, supplemented with manual research. The following keywords were searched in the databases in all possible combinations: Nintedanib; Pirfenidone, interstitial lung disease, and idiopathic pulmonary fibrosis. The most widely accepted method for evaluating the progression of ILD is through the decline in forced vital capacity (FVC), as determined by respiratory function tests. Specifically, a decrease in FVC over a 6–12-month period correlates directly with increased mortality rates. Antifibrotic drugs Pirfenidone and Nintedanib have been extensively validated; however, some patients reported several side effects, predominantly gastrointestinal symptoms (such as diarrhea, dyspepsia, and vomiting), as well as photosensitivity and skin rashes, particularly associated with Pirfenidone. In cases where the side effects are extremely severe and are more threatening than the disease itself, the treatment has to be discontinued. However, further research is needed to optimize the use of antifibrotic agents in patients with PF-ILDs, which could slow disease progression and decrease all-cause mortality. Finally, other studies are requested to establish the treatments that can stop ILD progression.

In recent years, there has been a growing recognition within the medical community that idiopathic pulmonary fibrosis (IPF) cannot be effectively managed through a singular focus on the disease itself. Instead, a dual approach is essential, one that not only aims to prolong survival by targeting the underlying pathological mechanisms of IPF but also addresses the numerous comorbidities that frequently complicate the clinical picture for affected individuals. This narrative review seeks to provide a detailed and comprehensive exploration of the various comorbid conditions associated with IPF, which may include cardiovascular disease (CVD), lung cancer (LC), gastroesophageal reflux disease (GERD), obstructive sleep apnea (OSA), and anxiety/depression, among others. By understanding the interplay between these comorbidities and IPF, healthcare providers can better tailor treatment regimens to meet the holistic needs of patients. Furthermore, this review delves into both current management strategies and emerging therapeutic approaches for these comorbidities, emphasizing the importance of interdisciplinary collaboration in clinical practice. By synthesizing the latest research and clinical insights, this review aims to enhance awareness and understanding of the complexities surrounding IPF management, ultimately guiding clinicians in developing more effective, individualized care plans that address not only the fibrotic lung disease but also the broader spectrum of health challenges faced by patients. Through this comprehensive overview, we hope to contribute to the ongoing dialogue about improving quality of life and survival rates for individuals living with IPF.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease of unknown aetiology. Evidence on the progression of idiopathic pulmonary fibrosis (IPF) following the introduction of antifibrotic therapies still indicates a generally poor prognosis. IPF is associated with both respiratory and non-respiratory comorbidities, which can worsen symptoms and impact overall survival. Background/Objectives: The study aimed to investigate the effect of these comorbidities on the early and permanent discontinuation of pirfenidone or nintedanib in IPF patients. Methods: In this single-centre retrospective study, 101 patients diagnosed with IPF according to ATS/ERS/JRS/ALAT guidelines were treated with AFT. Clinical data were collected at 12 months prior to and up to 24 months following treatment initiation, including age, gender, smoking history, and the presence of respiratory and non-respiratory comorbidities. Results: The data showed that 21 patients (20.8%) discontinued treatment within the first 12 months. Additionally, pre-treatment comorbidities were not statistically correlated with the suspension of antifibrotic treatment. Among the overall cohort, 77 patients (76.2%) had at least one comorbidity and 27 (26.7%) had three or more comorbidities. Notably, 24 (23.8%) had respiratory comorbidities, while 75 (74.3%) had non-respiratory comorbidities. Conclusions: This real-life study emphasises the complexities involved in managing IPF, particularly regarding adherence to treatment when significant comorbidities are present. The evidence suggests that in patients with IPF, pre-treatment respiratory or non-respiratory conditions do not affect AFT discontinuation.

Bronchiectasis has been reported in several systemic autoimmune rheumatic diseases, but published estimates are difficult to interpret because many studies count any bronchial dilatation on computed tomography (CT). This is particularly problematic in connective tissue disease-associated interstitial lung disease (ILD), where traction can enlarge the airways without producing the clinical syndrome of bronchiectasis. We reviewed evidence in rheumatoid arthritis, Sjögren disease, anti-neutrophil cytoplasmic antibody-associated vasculitis, systemic sclerosis, systemic lupus erythematosus, and inflammatory myopathies, with attention to imaging definitions, respiratory symptoms, infection, outcomes, and treatment. The association with a distinct airway disorder is best established in rheumatoid arthritis, Sjögren disease, and myeloperoxidase-ANCA-positive vasculitis. In systemic sclerosis and systemic lupus erythematosus, large administrative cohorts suggest an increased occurrence of bronchiectasis, but most data do not allow for confident separation from traction bronchiectasis or other causes. In inflammatory myopathies, reported bronchial dilatation is mainly described within fibrotic ILD. In practice, interpretation should combine high-resolution CT morphology and distribution with cough and sputum, exacerbation history, microbiology, and the presence and extent of ILD; traction and non-traction changes may coexist. Bronchiectasis-specific care should follow established guidelines, while immunomodulatory therapy is selected for the rheumatic disease with explicit consideration of infection risk. No trial has identified a preferred antirheumatic regimen for patients with bronchiectasis, and it remains unknown whether better control of systemic disease changes bronchiectasis outcomes. Studies using consistent clinical and radiological definitions are needed. In mixed connective tissue disease, available evidence is insufficient to characterise bronchiectasis independently of ILD. Seven disease categories are reviewed in total.

Background/Objectives: Telemedicine (TM) has become an increasingly important tool in the management of respiratory diseases, offering the potential to improve access to care, support long-term monitoring, and reduce the burden of in-person visits. This narrative review aims to critically summarize the current evidence on the achievements and limitations of TM across major areas of respiratory medicine. Methods: A non-systematic search of PubMed and Scopus was performed for English-language studies published up to April 2026, using combinations of keywords related to TM, telemonitoring, and respiratory diseases. Relevant articles were selected based on their clinical relevance and contribution to understanding the role of TM in diagnosis, monitoring, and management, and additional studies were identified through manual screening of reference lists. Results: The TM has shown consistent benefit for objective, device-derived outcomes—most notably CPAP adherence in obstructive sleep apnea—and for process outcomes such as reduced travel burden and improved cost-effectiveness primarily demonstrated in obstructive sleep apnea and COPD, with limited systematic evidence of cost-effectiveness in interstitial lung disease, home mechanical ventilation, cystic fibrosis, and smoking cessation. However, evidence for hard clinical endpoints such as hospitalization and mortality remains inconsistent, and successful implementation depends heavily on the surrounding care model rather than on technology alone. Conclusions: The TM represents a valuable complement to conventional respiratory care with demonstrated achievements in adherence, patient satisfaction, and healthcare efficiency, but its adoption remains limited by heterogeneous evidence, workforce and resource constraints, and the risk of widening digital disparities. Larger, adequately powered trials with harmonized outcomes and sustained investment in integrated care pathways are needed to fully realize its potential.