Rare ILD & eurILDreg Kick-off-meeting

22.06. – 24.06.2022 – Castle Rauischholzhausen

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Fibrosing lung diseases are accompanied by partly reversible and partly irreversible changes in lung tissue. The aim of treatment is therefore to cause the reversible changes to regress and to halt the irreversible changes.

Cortisone drugs are most frequently used in the treatment of fibrosing lung disease. These drugs have been successfully used in certain forms of sarcoidosis. In extrinsic allergic alveolitis, these drugs bring about accelerated regression of changes in the initial stages. If the lung fibrosis occurs within the context of rheumatic diseases, the cortisone therapy often needs to be augmented or replaced by other immunosuppressant drugs.

Based on previous recommendations cortisone and immunosuppressants (e.g. cyclophosphamide and azathioprine) have been frequently used for the treatment of patients with idiopathic pulmonary fibrosis (IPF). While the response to this treatment is somewhat rare and usually only means temporary stabilisation of the course and the use of azathioprine is meanwhile proven potentially harmful this treatment is no longer recommended.

To date the only anifibrotic substance that holds a market authorization within the EU is Pirfenidone. Pirfenidone is used for the treatment in mild to moderate IPF. Against this background, other medicines are currently being tested in clinical studies[D1] .

In addition to drug treatment, all patients benefit from stopping smoking, since smoking is associated with more rapid disease progression. Furthermore, despite the lack of studies on the benefit of physical exercise, the experts are unanimous that regular exercise improves the symptoms and quality of life. Infections of the airways should be treated early and generally with antibiotics. In the absence of a response, inpatient treatment is usually necessary.

Depending on more specific circumstances, the possibilities and risks of a lung transplantation should be discussed when IPF is diagnosed so as to be able to undertake the necessary preliminary examinations early on, for example.

Given the poor prognosis of IPF, alternative treatment approaches have recently been developed. Studies on these have been and are being conducted:

Phase II studies    Phase III studies (ongoing or completed) 
  • inhaled heparin (DOSEFIB)
  • the leukotriene antagonist zileutin
  • antibodies to CTGF (connective tissue growth factor)
  • the TNF-α inhibitor etanercept
 
  • N-acetylcysteine (IFIGENIA)
  • Interferon-γ 1b (INSPIRE)
  • Pirfenidone (CAPACITY)
  • Glivec (imatinib mesylate)
  • Bosentan, Macitentan (BUILD 3, MUSIC)
  • Intedanib (BIBF1120)
  • Ambrisentan (ARTEMIS)
  • Sildenafil (STEP-IPF) 

In recent years, much new information has been obtained on how IPF occurs. Developing new treatments based on these findings and being able to offer these to patients will be a challenge of the next few years.

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