Interstitial and Diffuse Pneumonias Clinic

What are interstitial pneumonias?

Diffuse interstitial pneumonias are a group of relatively rare lung diseases. They are disorders of the lung parenchyma (that is, of the lung itself) with a varying degree of inflammation and fibrosis resulting in a sometimes severe respiratory insufficiency. 

The principal pathologies covered by this term are

•    Idiopathic pulmonary fibrosis
•    Sarcoidosis
•    Non-specific interstitial pneumonia
•    Cryptogenic organising pneumonia
•    Lung disorders secondary to autoimmune diseases (connective tissue disease and vasculitis) 

•    Hypersensitivity pneumonitis (farmer‘s lung, bird fancier’s lung

•    Some pneumonias of occupational origin (exposure to asbestos or to mineral or metal dust) or drug induced

Idiopathic pulmonary fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is the most common disease of this group, together with sarcoidosis. The annual incidence is estimated at 10 new cases for 100,000 persons per year and this figure seems to be rising. In IPF the lung is progressively destroyed and replaced by scar tissue. This results in an irreversible and progressive respiratory insufficiency. 

Causes and risk factors

Its etiology is unknown but smoking, pollution, exposure to certain viruses and the presence of gastroesophageal reflux are risk factors. There are also family forms in which gene mutations have been clearly identified.  IPF affects more men than women and is generally diagnosed at around the age of 60. 

Diagnosis and treatment

These diseases are rare and therefore demand particular expertise. The diagnosis is complex and requires a multidisciplinary discussion.  In the case of idiopathic pulmonary fibrosis there are two possible treatments: with Esbriet or Ofev. These do not cure the diseases but do make it possible to slow its progress. 

If the patient shows no contraindications a lung transplant must be envisaged. In this particular case we work closely together with the lung transplant team. Multidisciplinary meetings are held regularly to discuss complex cases and we have a clinical research unit that permits access to an advanced research programme in this field and patients are invited to participate in international trials to test new treatments. 

The Erasmus Hospital is a member of the European experts network in the field of diffuse interstitial pneumonia (ERN lung). We are active in the field of clinical research by providing the opportunity to participate in research protocols and clinical trials. 

Finally, we support and participate actively in activities organised by patients’ associations, in particular the   Association Belge Francophone contre la Fibrose Pulmonaire (ABFFP).

Requests for opinion

Téléchargez le document de demande d'avis de la part du médecin traitant ou spécialiste à destination de la Clinique des pneumopathies interstitielles diffuses.

Multidisciplinary team

Découvrez l'équipe multidisciplinaire de la Clinique de Pneumopathies interstitielles et diffuses.

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