Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal disease. The treatment is challenging and nowadays a comprehensive approach based not only in pharmacological strategies is necessary. Identification and control of comorbidities, non-pharmacological treatment, prevention and management of exacerbations as well as other areas of care (social, psychological) are fundamental for a holistic management of IPF. Gastroesophageal reflux, pulmonary hypertension, obstructive sleep apnea, combined with emphysema, lung cancer and cardiovascular involvement are the main comorbidities associated with IPF. Non-pharmacological treatment includes the use of oxygen in patients with rest or nocturnal hypoxemia and other support therapies such as non-invasive ventilation or even a high-flow nasal cannula to improve dyspnea. In some patients, lung transplant should be considered as this enhances survival. Pulmonary rehabilitation can add benefits in outcomes such control of dyspnea, exercise capacity distance and, overall, improve the quality of life; therefore it should be considered in patients with IPF. Also, multidisciplinary palliative care programs could help with symptom control and psychological support, with the aim of maintaining quality of life during the whole process of the disease. This review intends to provide clear information to help those involved in IPF follow up to improve patients’ daily care.

Immune checkpoint inhibitors (ICIs) have transformed cancer therapy, offering significant efficacy and a generally favorable safety profile. However, they are associated with a spectrum of immune-related adverse events (irAEs), among which pneumonitis stands out due to its relatively high mortality. This condition is characterized by nonspecific clinical manifestations and a broad differential diagnosis, often requiring the involvement of pulmonologists for accurate diagnosis and management. Given its potential severity, it is crucial for pulmonologists to be well-versed in recognizing and addressing checkpoint inhibitor pneumonitis (CIP). In this narrative review, we examine reported cases of pneumonitis in patients with various types of cancer treated with ICIs. The article explores the mechanisms of action of ICIs, the underlying pathophysiology of pneumonitis, associated risk factors, clinical presentation, diagnostic approaches, and current treatment strategies, intended to support pulmonologists in improving early detection and implementing evidence-based management of this potentially life-threatening complication.

Immune checkpoint inhibitors (ICIs) improve cancer outcomes but can cause severe immune-related adverse events, including pneumonitis, which is associated with high mortality and a non-specific clinical presentation. We aimed to identify factors associated with ICI-related pneumonitis. We performed a multicentre retrospective case–control study in five tertiary hospitals in Spain. Patients diagnosed with pneumonitis between 2017 and 2025 were matched with controls by ICI and underlying malignancy. Clinical, radiological, bronchoscopic, treatment and outcome data were analysed. Overall, 111 patients were included; 60% had lung cancer and 74% were male. Grade III–IV pneumonitis occurred in 52%. Ground-glass opacities were the most common CT finding (71%), with organising pneumonia as the predominant pattern (42%). Bronchoscopy was performed in 66%, showing lymphocytosis in 71% of bronchoalveolar lavage samples. ICIs were discontinued and systemic corticosteroids initiated in 92% of patients; 14% required additional immunosuppression. Pneumonitis resolved in 55%, relapsed in 10%, and attributable mortality was 10%. In the matched case–control analysis (106 cases and 106 controls), no statistically significant associations were identified. erj;68/suppl_70/PA6284/TB1 T1 TB1 Variable Main result Smoking status (ever vs never) OR 1.36 (95% CI 0.68–2.69), p = 0.39 Pre-existing lung disease OR 1.22 (95% CI 0.70–2.13), p = 0.48 Allergy history p = 0.23 Prior chemotherapy OR 1.01 (95% CI 0.54–1.91), p = 0.97 Baseline FVC (L) OR 0.90 (95% CI 0.76–1.08), p = 0.27 ECOG performance status ≥1 p > 0.05 Extrapulmonary irAEs OR 0.68 (95% CI 0.35–1.31), p = 0.24 ^{OR: odds ratio; CI: confidence interval; L: litres; irAEs: immune-related adverse events; ECOG: Eastern Cooperative Oncology Group performance status.}