Interstitial and rare lung diseases

Interstitial lung diseases is a group of lung diseases that mainly affect the alveolar space (air sacs of the lungs) and the tissue between the air sacs (interstitium). In interstitial lung disease, repair processes of the lungs are impaired and the tissue around the air sacs (alveoli) becomes fibrotic (scarred).


Typically, the first symptoms are not very specific. Cough and dyspnea are early symptoms but sometimes it takes years until a definite diagnosis can be established. An advanced disease stage is associated with an impaired quality of life, a low exercise capacity and a reduced survival.


Many different triggers are known that may cause lung fibrosis. We distinguish between interstitial lung disease of known causes (e.g. certain medication or in the context of rheumatological diseases) and lung fibrosis of unknown (idiopathic) cause.


High resolution CT of the chest is the preferred radiologic modality and pulmonary function tests allow to quantify impair functional impairment. A lung biopsy is required if clinical history and imaging are not clearly suggestive of a certain diagnosis.


If a specific occupational exposure is found or a responsible drug is suspected, avoidance of specific triggers is important. Treatment differs among the disease entities and encompasses either an anti-inflammatory or antiproliferative treatment. Lung transplantation is a option if lung fibrosis progresses and does not respond to the therapeutic efforts.


Patient with interstitial lung diseases are usually discussed with several experts and in cooperation with the department of radiology and pathology and sometimes with our colleagues from the rheumatology or immunology service. Patient can be referred to the ILD-board ( In this setting diagnostic procedures and therapeutic strategies are discussed and defined. We also have the opportunity to discuss cases on a video-conference system.

Spectrum of diseases:

  • Idiopathic pulmonary fibrosis (IPF)
  • Non-specific interstitial pneumonia (NSIP)
  • Cryptogenic organizing pneumonia (COP)
  • Lympholymphocytic interstitial pneumonia (LIP)
  • Smoking-associated interstitial lung disease (RB-ILD, DIP)
  • Lung diseases in context of connective tissue diseases and other rheumatological disorders
  • Sarkoidosis
  • Osler-Rendu-Weber-disease
  • Silicosis
  • Hypersensitivity pneumonitis
  • Cystic lung disease e.g.:
  • Common variable immundeficiency (CVID)
Last update: 11.07.2019 | Person responsible:
Professor Malcolm Kohler, MD

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