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Interest in the role played by comorbidities in IPF has increased in the past few years. The optimal clinical management of IPF is multifaceted and not only involves antifibrotic treatment, but also vaccinations, oxygen supplementation, evaluation of nutritional status as well as psychological support and patient education. Symptom management, pulmonary rehabilitation, palliative care and treatment of comorbidities represent further areas of clinical intervention. This review analyses the major comorbidities observed in IPF, focusing on those that have the greatest impact on mortality and quality of life (QoL). The identification and treatment of comorbidities may help to improve patients' health-related QoL (<jats:italic>i.e.<\/jats:italic>sleep apnoea and depression), while some comorbidities (<jats:italic>i.e.<\/jats:italic>lung cancer, cardiovascular diseases and pulmonary hypertension) influence survival. 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