Definition
Pulmonary fibrosis is a respiratory disease characterized by the formation of scar tissue that replaces normal lung tissue.
The scarring causes a hardening of the lungs, forcing the alveoli and thus hindering normal breathing.
There are basically two types of pulmonary fibrosis: idiopathic and secondary.
Causes
As far as idiopathic pulmonary fibrosis is concerned - as its name suggests - at the moment, a real triggering cause has not yet been identified.
As regards secondary pulmonary fibrosis, however, there are several possible causes that favor its development. Among these, we mention the exposure to toxic substances, some types of bacterial infections, the assumption of some types of anticancer drugs, radiotherapy, some drugs used for the treatment of heart diseases and some autoimmune diseases (such as systemic lupus erythematosus, sarcoidosis, rheumatoid arthritis, scleroderma and Wegener's granulomatosis).
In addition, elderly patients, smokers, patients undergoing lung or breast radiotherapy, patients taking cancer chemotherapy, workers in agricultural or metallurgical companies and individuals with a family history of pulmonary fibrosis, are subject to a greater risk of developing the disease.
Symptoms
The symptoms that patients with pulmonary fibrosis may experience are dyspnea, dry cough, chest pain, body weight loss, fatigue, weakness, muscle and joint pain.
Furthermore, cyanosis, edema, haemothorax, respiratory acidosis, hypertrophy of the accessory muscles of breathing, pneumothorax, rales, wheezing, hypoxia, water retention and drowsiness can also occur.
Finally, pulmonary fibrosis can favor the development of serious complications, such as respiratory failure, pulmonary hypertension, pulmonary heart and lung cancer.
Information on Pulmonary Fibrosis - Drugs and Care is not intended to replace the direct relationship between health professional and patient. Always consult your doctor and / or specialist before taking Pulmonary Fibrosis - Drugs and Care.
drugs
Unfortunately, there are no real drugs to treat pulmonary fibrosis, since the scar tissue is formed in a continuous and irreversible manner. The pharmacological treatment used for this pathology, therefore, aims to reduce the symptoms and slow down its progress, in an attempt to improve the quality of life of the patients who suffer from it.
The drugs that are usually used in the treatment of pulmonary fibrosis are, for the most part, corticosteroids and immunosuppressants.
Furthermore, to limit the symptoms induced by pulmonary fibrosis, oxygen therapy and respiratory rehabilitation can also be very useful.
In very serious cases and in cases where the aforementioned treatments are ineffective, however, the doctor may decide to intervene by practicing a lung transplant.
Furthermore, patients diagnosed with pulmonary fibrosis should make changes to their lifestyle, avoiding both active and passive smoking, adopting a balanced diet and carrying out an influenza vaccine and pneumonia vaccine, since - if contracted - these respiratory infections could further aggravate the symptoms induced by pulmonary fibrosis.
Corticosteroids
As mentioned, corticosteroids can be used in the treatment of pulmonary fibrosis to try to limit its symptoms. They are used because of their anti-inflammatory and immunosuppressive properties.
However, the use of these drugs must be carried out with caution and under the strict control of the doctor, due to the serious side effects they can cause.
Among the various steroidal anti-inflammatories that can be used in the treatment of pulmonary fibrosis, we recall the:
- Prednisone (Deltacortene ®): prednisone is available for oral administration. The usual drug dose is 5-15 mg a day. In any case, the exact quantity of prednisone to be taken must be established by the doctor according to the severity of the disease and according to the patient's response to the treatment.
immunosuppressant
Immunosuppressive drugs can be used in the treatment of pulmonary fibrosis, especially when it is caused by autoimmune diseases. In fact, these drugs are able to reduce the activity of the immune system.
Among the various active ingredients that can be used, we recall cyclosporine (Ciqorin ®, Sandimmun ®). It is a drug available in different pharmaceutical formulations suitable for different routes of administration, including oral and parenteral administration. The dose of active ingredient to be used must be established by the doctor on an individual basis for each patient.
Pirfenidone
Pirfenidone (Esbriet ®) is a relatively recent drug (its use in Europe, in fact, was approved only in 2011) and has specific therapeutic indications for the treatment of idiopathic pulmonary fibrosis.
It is a molecule with anti-inflammatory and anti-fibrotic activity, for these reasons it is particularly indicated for the treatment of this respiratory pathology.
Pirfenidone is available for oral administration as hard capsules.
Generally - during the first seven days of treatment - 267 mg of drug are administered three times a day with food.
Then - over the next seven days of treatment - the dose is increased to 534 mg of active ingredient, to be taken with food always three times a day.
From the fifteenth day of therapy onwards, however, the dose is further increased to 801 mg of pirfenidone three times a day, to be taken with food.
However, when starting the pirfenidone treatment, it is necessary to scrupulously follow all the indications provided by the doctor, both as regards the amount of drug to be taken, both as regards the frequency of administration and the duration of the same treatment.
