Comorbidities, Complications and Non-Pharmacologic Treatment in Idiopathic Pulmonary Fibrosis

Abstract

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.

Comprehensive Care in Pulmonary Fibrosis

“…On the other hand, ILD specialists need to better interact The main symptoms that drive QoL in IPF are cough, dyspnoea and anxiety 28 . Different treatment strategies have been used to relieve chronic cough and progressive dyspnoea associated with pulmonary fibrosis, presenting limited evidence and modest results 29,[40][41][42][43][44][45][46][47] .…”

“…Patients with mild or no symptoms and no limitations for daily life activities would need some form of supervision of their weekly physical activity or exercise, or, in case of sedentary habits, initiate them withina pulmonary rehabilitation program, while patients in more advanced stages could benefit from physiotherapy to improve patient's autonomy. The presence of debilitating comorbidities and their treatment may also interfere in the patient's physical activity and QoL 45 (Fig. 3).…”

“…The prevalence of lung cancer and obstructive sleep apnoea is higher than in the global population. Lung cancer, pulmonary hypertension and pulmonary embolism are associated with poor prognosis (reproduced and modified with permission from Millan-Billi P et al 45 ). COPD: chronic obstructive pulmonary disease; CPAP: continuous positive airway pressure; QT: chemotherapy; RDT: radiotherapy.…”

Guidelines of the Polish Respiratory Society for Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

“…There are no unequivocal recommendations for effective management both in the case of a known cause and an acute exacerbation in an IPF patients if the event is associated with life-threatening hypoxaemia. The choice of therapeutic methods is limited to invasive ventilation, non-invasive ventilation (NIV) support, extracorporeal membrane oxygenation (ECMO) or administration of oxygen through a high-flow nasal cannula (HFNC) [375]. Idiopatic pulmonary fibrosis patients admitted to intensive care units due to acute respiratory failure who require mechanical ventilation are burdened with a poor prognosis and high mortality [376].…”

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

“…Breathing rate and minute ventilation are decreased with HFNC [41]. In AE of IPF patients, pharrmacological treatment with HFNC can provide relief of dyspnea [42]. HFNC Future multi-center study will be anticipated.…”