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).
Symptoms
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.
Causes
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.
Diagnosis
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.
Therapy
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.
ILD-Board
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 (ild-board@usz.ch). 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)
