Pulmonary fibrosis – when the lungs become scarred

Pulmonary fibrosis – when the lungs become scarred

In pulmonary fibrosis, scar tissue progressively replaces the lung tissue, causing lung function to deteriorate gradually. Pulmonary fibrosis is, therefore, primarily noticeable through symptoms such as shortness of breath and a dry cough. Various diseases, infections or other harmful influences can cause fibrosis of the lungs. Often, however, no cause can be determined – this is referred to as idiopathic pulmonary fibrosis. The course, therapy and life expectancy depend on the form of pulmonary fibrosis and are, therefore, individually different for each patient. 

What is pulmonary fibrosis?

Lung fibrosis describes a process in the lungs in which there is a pathological proliferation of scarred connective tissue in the spaces between the cells (interstitium). As a result, the functional tissue – the alveoli – is gradually displaced, and the lung function progressively deteriorates.

It is caused by chronic inflammatory processes in the lungs, which various factors can trigger.

 

Pulmonary fibrosis: symptoms are dry cough and shortness of breath

The onset of pulmonary fibrosis is initially noticeable through shortness of breath during exertion – later, shortness of breath usually also occurs at rest. In addition, patients often complain of a dry, hacking cough. If necessary – for example, if an infection is the cause – fever can occur. 

In the advanced stage, changes in the hands can usually also be seen: Due to the chronic lack of oxygen, the nails (watch glass nails) bulge, and the fingertips (drumstick fingers) widen. The skin may also turn blue (cyanosis). 

Classification of fibrosis by cause

There are two main groups of pulmonary fibrosis: secondary and idiopathic pulmonary fibrosis. One speaks of secondary pulmonary fibrosis when a cause for the fibrotic lung change can be identified. 

The following diseases and factors are possible triggers for such secondary pulmonary fibrosis:

  • Infections, for example, with viruses, the fungus Pneumocystis jirovecii or legionella
  • Autoimmune diseases such as sarcoidosis
  • Diseases of the connective tissue ( collagenosis ) such as scleroderma, systemic lupus erythematosus or Sjögren’s syndrome
  • Rheumatic diseases such as chronic polyarthritis or vascular inflammation (vasculitis)
  • Lung diseases caused by inhaled dust – such as asbestos, quartz dust or metals (pneumoconiosis) or fine dust (exogenous allergic alveolitis)
  • inhaled pollutants such as gases, vapours or tobacco smoke
  • certain medications (such as chemotherapy drugs) 
  • ionizing radiation,  such as after radiation therapy
  • Diseases that cause chronic excess fluid in the lungs pulmonary oedema), such as heart failure or kidney failure
  • specific forms of lung cancer (bronchioloalveolar carcinoma)

In about 50 per cent of cases, however, no cause for pulmonary fibrosis can be found. It is then referred to as “idiopathic interstitial pneumonia” (IIP), which means something like “pneumonia of the intercellular spaces of unknown cause”. 

 

Risk factors for idiopathic pulmonary fibrosis

Within the group of idiopathic pulmonary fibrosis, there are different sub-forms. The most common of these is what is known as idiopathic pulmonary fibrosis (IPF). 

Although no cause can be found in idiopathic pulmonary fibrosis by definition, studies indicate some possible risk factors. Influences that can increase the risk of the disease include:

  • Smoking
  • Environmental pollution, such as metal dust, plant dust and livestock dust
  • Infections, such as the Epstein-Barr virus or hepatitis C virus
  • frequent heartburn (reflux disease)
  • Diabetes mellitus
  • genetic factors

Diagnostics: find possible causes

To be able to diagnose secondary or idiopathic pulmonary fibrosis, the doctor must first question the patient in detail about possible risk factors, previous illnesses, occupation and other influences. After a physical examination, a lung function test is usually performed. 

blood test can estimate the severity of the lung damage based on the oxygen content in the blood. In addition, specific blood values or markers can indicate possible causes of pulmonary fibrosis. 

CT as a critical diagnosis

Computed tomography (CT) plays a vital role in diagnosis. As a rule, a so-called high-resolution CT (HR-CT) is performed if pulmonary fibrosis is suspected – it offers a higher resolution than conventional CT. It can, therefore, show the changes in the lungs particularly well. 

A typical pattern for idiopathic pulmonary fibrosis is called “Usual Interstitial Pneumonia (UIP)”. If this can be determined with certainty, the IPF diagnosis is secured. In all other cases, a tissue sample from the lungs (biopsy) may need to be taken. A so-called bronchoalveolar lavage carried out through a lung endoscopy can also be helpful under certain circumstances. 

 

Idiopathic pulmonary fibrosis: drugs show little promise

In treating secondary pulmonary fibrosis, the focus is on treating the cause or avoiding triggering factors. The treatment of idiopathic pulmonary fibrosis, on the other hand, is currently still the subject of research: numerous studies have tested the effectiveness of various drugs, but so far, no promising drug therapy has been found (as of October 2016). 

Under certain circumstances, treatment with drugs that suppress the immune system (immunosuppressants) – possibly in combination with cortisone-like active ingredients (corticoids) – can lead to an improvement. According to recent studies, the active ingredient pirfenidone can slow the progression of pulmonary fibrosis. 

Pulmonary rehabilitation as a therapy option

In the absence of practical drug therapy options, non-drug treatment is of great importance in idiopathic pulmonary fibrosis. There are special rehabilitation programs for this (so-called pulmonary rehabilitation), which, in addition to training, psychological care and nutritional advice, also include moderate strength and endurance training. 

Oxygen therapy can relieve symptoms.

If the lung function continues to deteriorate, the administration of oxygen can alleviate the symptoms and increase performance. Such oxygen therapy is also possible at home and on the go using a nasal cannula and a mobile oxygen cylinder. 

In addition, all patients with pulmonary fibrosis should quit smoking and receive influenza and pneumococcal vaccinations to prevent infection.

 

Lung transplantation: The last option in the final stages

Pulmonary fibrosis cannot be cured because the scarring of the lungs cannot be reversed. Therefore, treatment aims to slow down the progression of the disease and improve quality of life.

Lung transplantation can, therefore, be considered in end-stage idiopathic pulmonary fibrosis. However, many patients are unsuitable for a transplant due to comorbidities or being too old. 

Life expectancy: How long do patients with pulmonary fibrosis live?

Idiopathic pulmonary fibrosis is usually progressive, whereas the secondary form can resolve if the cause is successfully treated. 

Therefore, the course and prognosis of the disease are individually different for each patient, and it is impossible to make a general statement about life expectancy. 

However, the average survival time from the diagnosis of pulmonary fibrosis is two to five years.

pulmonary fibrosis and COPD

The so-called obstructive lung disease, in which the lung function is not reduced by scarring but by a narrowing or obstruction of the airways – for example, due to mucus – must be distinguished from pulmonary fibrosis. 

The best-known form is chronic obstructive pulmonary disease ( COPD ). The hereditary disease cystic fibrosis (also misleadingly referred to as cystic fibrosis) is also obstructive because the airways are blocked by viscous mucus.

Pulmonary fibrosis, on the other hand, is referred to as a restrictive lung disease because it can no longer develop appropriately due to scarring and shrinks. However, it is not uncommon for COPD patients to also have pulmonary fibrosis – possibly due to the shared risk factor of tobacco smoke.

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