Researchers from Vall d'Hebron University Hospital and the pneumology group of Vall d'Hebron Research Institute (VHIR) have demonstrated that the causes of idiopathic pulmonary fibrosis can be determined in half of the cases after an in-depth clinical study, which includes an exhaustive and systematized interrogation, determination of antibodies against the causative substances, inhalation tests, cultures and environmental measurements in the places frequented by patients (home and work) to detect antigens that may cause this serious disease.

"The importance of this clinical study, carried out with patients from the outpatient clinic of the Hospital's Pneumology Service, is that it demonstrates that with systematized, in-depth care and the right techniques, advances can also be achieved for our healthcare system," says Dr. Ferran Morell, lead author of the study.

The study, published in The Lancet Respiratory Medicine, was conducted between 2004 and 2011 in 60 patients with the disease and opens a new avenue in the diagnosis and treatment of this pathology, since the identification of the causes makes it possible to prevent it and thus prevent it from progressing to advanced or severe stages.

Researchers have discovered that one of the main causes of idiopathic pulmonary fibrosis is exposure to feather comforters and pillows, as well as exposure to birds and fungi in minimal but persistent quantities. In short, it is a disease called chronic hypersensitivity pneumonitis, a disease in which the Vall d'Hebron University Hospital is a world reference.

In order to diagnose this pathology in time, Dr. Ferran Morell assures that "it is essential for the diagnostic study of patients to have techniques for the detection of antibodies against the causal substances and inhalation tests (bronchoprovocation), as well as the realization of soluble extracts from the substances, etc.".

A new approach to the diagnosis and treatment of idiopathic pulmonary fibrosis

This study radically changes the approach to be taken in the study of the disease. In order to determine the causes of idiopathic pulmonary fibrosis, the pulmonologist must take a very precise and targeted history, including visits to the patient's home or workplace to try to discover possible antigenic sources. During the home/work visit, measurements of these antigens (often fungi, feathers, isocyanates, etc.) are even taken in the ambient air. From the suspected material, an antigenic extract is obtained in the pneumology research laboratory to determine whether there are specific IgG antibodies to this extract in the patient's serum (usually fungi, feathers, etc.). Simultaneously, a fungal culture of the suspected material is performed. With all this we obtain a good starting point to begin an in-depth study aimed at certifying the cause that has conditioned the patient to develop the disease.

After performing the immunological tests, a bronchofibroscopy with lavage study (secretions) and even a cryobiopsy are indicated. Less frequently, a surgical biopsy is performed to extract a sample of lung tissue and check the characteristic alterations of this pathology.

Finally, an inhalation test is performed with the antigen suspected of causing chronic hypersensitivity pneumonitis, a disease in which the Pneumology Department of the Vall d'Hebron University Hospital is a world reference.

A serious disease with low survival

Idiopathic pulmonary fibrosis is a disease recognized since 1940, in which both lungs progressively become fibrotic (scarred). In this way, the lung loses elasticity, which makes inspiration difficult and the patient gradually loses the ability to breathe. This disease affects about 10,000 patients in Spain (2,000 in Catalonia) and has an incidence of between 10 and 20 new cases per 100,000 inhabitants per year. In the last ten years several antifibrotic drugs have been tested with the aim of significantly increasing patient survival, which is only a few years. Lung transplantation is a solution for those who meet the criteria for surgery.

The first lung transplant performed successfully in Spain was carried out at the Vall d'Hebron University Hospital on an 18-year-old patient with this pathology, precisely because she had cared for a parrot. Thirty percent of the lung transplants performed at Vall d'Hebron Hospital correspond to patients with pulmonary fibrosis who had progressed to an advanced stage of the disease. In 2012 alone, 66 lung transplants were performed at Vall d'Hebron, a figure that places it among the top centers worldwide.