Diagnosis to disease management

A confirmatory ILD diagnosis is important for the initiation of the appropriate treatment. Those available pharmacological treatments, while not curative, can provide patients with some relief from ILD symptoms and help improve their overall quality of life [4–6]. Other non-pharmacological options for ILD include pulmonary rehabilitation and oxygen therapy. One study found that over 40% of patients who attended a specialist ILD clinic were referred for pulmonary rehabilitation [7]. Pulmonary rehabilitation can improve exercise capacity, quality of life and lung disease knowledge in ILD patients [7].

As ILD progresses many patients begin to experience declines in lung function, worsening disease symptoms and a lower quality of life [8]. Consequently, ILD patients are required to attend regular clinic follow-up appointments. These follow-up appointments typically involve the patient undergoing repeat imaging and pulmonary function testing [9]. However, many patients with advanced ILD report challenges in being able to both travel to and attend clinic appointments and perform pulmonary function tests e.g. spirometry due to their worsening respiratory symptoms  [10].

Some ILD patients may also experience acute exacerbations characterised by the rapid onset of breathlessness, coughing, fatigue and sometimes fever. ILD exacerbations may occur because of disease progression or may be triggered by infections, gastroesophageal reflux disease, air pollution or certain medications. It is estimated that 4% of IPF patients experience an acute exacerbation each year [11]. ILD patients who experience exacerbations may be admitted to hospital where they receive antibiotics and supportive care. Approximately half of all ILD patients who suffer an exacerbation do not survive to discharge [11].

The end of the journey

At the end of their journey, ILD patients experience significant escalations in disease symptoms and declines in functional capacity. Some ILD patients may undergo a lung transplant of either one or both lungs. Lung transplants can be life-saving for ILD patients with a median survival of around 7 years [12]. The procedure itself is however not without risk with an estimated 20% of patients not surviving 1-year post-transplant [13]. The long-term prognosis for ILD patients who do not undergo lung transplantation is significantly poorer with a median survival of 3 to 4 years post-diagnosis [14]. Consequently, many ILD patients spend the last few months of their life in a hospital or hospice facility [15].

In conclusion, the journey for many ILD patients is a difficult one fraught with many challenges. These challenges include inaccurate and delayed diagnoses, limited treatment options with the ability to halt ILD progression and poor patient monitoring. Addressing these challenges will be key to optimising patient outcomes and improving the quality of life of ILD sufferers.