Specialised respiratory conditions

Despite the heterogeneity of this group of diseases, in most of them, damage to the cells that line the air sacs (or ‘alveoli’) of the lungs leads to the formation of scar tissue which is an essential response of the body against pathogens and in normal wound healing. This makes it harder for oxygen to pass into the bloodstream, and as a result, the brain, heart and other vital organs may not get the amount of oxygen they need to work properly. The amount of scarring usually increases over time, although how quickly it progresses varies¹.

The symptoms develop gradually and can differ significantly from person to person. At the time of diagnosis, the most common symptoms include shortness of breath, a persistent dry cough, and fatigue. As the disease progresses, the cough may become productive, and later stage symptoms can include loss of appetite, aching joints and muscles, and gradual, unexplained weight loss. When a healthcare professional listens to the lungs of a patient through a stethoscope, they may detect a distinctive crackling sound, often described as similar to the sound of tearing velcro. These sounds are produced by the small airways opening during inhalation and are typically heard in both lungs¹.

Diagnosing pulmonary fibrosis is a multi-step process that typically begins with a CT scan of the lungs, followed by further investigations such as lung function testing, bronchoalveolar lavage, blood tests, or a biopsy. Where fibrotic interstitial lung disease (ILD) is suspected, a multidisciplinary team including a pulmonologist, radiologist, and pathologist, ideally supported by a specialist nurse, should confirm the diagnosis¹.

Decisions about pharmacological treatments depend on the diagnosis and the disease course. Antifibrotic drugs are recommended for pulmonary fibrosis, as they help to prevent tissue scarring and have been shown to slow progression of the disease over time and may increase life expectancy. For some patients, immunomodulation (substances that affect the functioning of the immune system) is generally recommended as initial treatment. If the disease still progresses, antifibrotic therapy can be considered. Besides drugs, there are a number of non-pharmacological treatments that can enhance patients’ quality of life, such as pulmonary rehabilitation, oxygen therapy or lung transplantation¹.