Symptom-based management of the idiopathic interstitial pneumonia.

The term 'idiopathic interstitial pneumonia' (IIP) encompasses a wide variety of diseases with different and often unexplained pathophysiology as well as diverse natural histories. Unfortunately, many of these diseases are progressive and some are poorly responsive to available therapies. Despite the varied nature of IIPs, patients experience common symptoms related to their chronic lung disease. Dyspnoea, cough, fatigue and depression contribute substantially to morbidity and are often difficult to manage. The psychological stress of having a chronic and often life-limiting disease further complicates symptom control. Effective symptom-management requires a multidisciplinary approach that incorporates patient education and self-management to formulate goals of care and treatment plans. In this context, palliative care is incorporated from the time of diagnosis of an IIP and is not restricted to the end stages of the disease. Pulmonary rehabilitation plays a central role in symptom-management and has beneficial effects across multiple domains. In patients who do not respond to disease-specific treatments and are not candidates for lung transplant, early referral to hospice may improve quality of life for both patients and their families near the end of life.

Lung transplantation in IIP: A review.

The idiopathic interstitial pneumonias (IIP) encompass a large and diverse subtype of interstitial lung disease (ILD) with idiopathic pulmonary fibrosis (IPF) and non-specific interstitial pneumonia (NSIP) being the most common types. Although pharmacologic treatments are available for most types of IIP, many patients progress to advanced lung disease and require lung transplantation. Close monitoring with serial functional and radiographic tests for disease progression coupled with early referral for lung transplantation are of great importance in the management of patients with IIP. Both single and bilateral lung transplantation are acceptable procedures for IIP. Procedure selection is a complex decision influenced by multiple factors related to patient, donor and transplant centre. While single lung transplant may reduce waitlist time and mortality, the long-term outcomes after bilateral lung transplantation may be slightly superior. There are numerous complications following lung transplantation including primary graft dysfunction, chronic lung allograft dysfunction (CLAD), infections, gastroesophageal reflux disease (GERD) and airway disease that limit post-transplant longevity. The median survival after lung transplantation is 4.7 years in patients with ILD, which is less than in patients with other underlying lung diseases. Although long-term survival is limited, this intervention still conveys a survival benefit and improved quality of life in suitable IIP patients with advanced lung disease and chronic hypoxemic respiratory failure.