Managing idiopathic pulmonary fibrosis in elderly patients

  • Feb 07 , 2017

Idiopathic pulmonary fibrosis (IPF) is a progressive disease related to the lung. It is a type of interstitial lung disease, which is a group of 200 diseases with similar symptoms but different causes. In IPF, lung’s tissue becomes damaged. The scarring typically starts at the edges of the lungs and progresses towards the centre of the lungs, making it more and more difficult for a person to breathe. Unfortunately, IPF is a disabling disease without a known cure or treatment.

IPF mostly affects middle-aged and older adults, and there is no cure. The progress of the disease varies from person to person, but often people diagnosed with idiopathic pulmonary fibrosis live about three to five years following their diagnosis. While the cause of IPF is unknown, it is thought that genetics may be a factor. When more than one member of a family has IPF, the disease is referred to as familial idiopathic pulmonary fibrosis.

Symptoms of IPF

  • In the disease, patients often complain of a dry and unexplained cough.
  • Shortness of breath – With time, shortness of breath (dyspnea) worsens. Dyspnea initially occurs only with activity and is often attributed to ageing. Over time, the dyspnea occurs with little or no activity. Eventually, the shortness of breath becomes disabling, limiting all activity and even occurring while sitting still.
  • In some cases, the fibrosis can be rapidly progressive, dyspnea and disability occurring in weeks to months from the onset of the disease. This form of pulmonary fibrosis has been referred to as Hamman-Rich syndrome.

Management of IPF

  • Idiopathic pulmonary fibrosis is strongly associated with old age. In an ageing population, lung disease has become a major cause of morbidity and mortality worldwide. IPF is the 4th leading cause of death.
  • Making an accurate diagnosis of IPF is tough, as it remains only one of many potential diagnoses for an elderly patient with newly recognised interstitial lung disease. Optimal management of IPF, especially in older-aged patients, rely on such factors as balancing standard of care of measures given to the patient’s overall health status and considering the patient’s wishes, desires, and expectations.
  • IPF is known to be associated with certain comorbidities that tend to be more prevalent in the elderly people. Until recently, options for the pharmacologic management of IPF were limited and included therapies such as immunosuppressive agents, which may pose a substantial risk to the elderly patient. However, anti-fibrotic agents have now become available.
  • The monitoring and treatment of patients with IPF, especially elderly patients with comorbid medical conditions, require consideration of adverse side effects, the avoidance of potential drug-drug interactions, treatment of comorbidities, and the timely implementation of supportive and palliative measures. Individualised counselling to guide decision-making and enhance the quality of life is also integral to the optimal management of the elderly patient with IPF.