Fibrosis Pulmonar

Pulmonary fibrosis

The objective of this blog is to help the general population learn about various diseases related to Pulmonology. In such a way that anyone who suffers from a respiratory disease can know it better or can be able to promptly identify any symptoms that require medical evaluation and that this allows them to make broadly informed decisions with their doctor.

At the end of the post you can leave us your comments and questions, or if you wish directly to our email: neumologo.oc@breathbaja.com

 
 

Let's define pulmonary fibrosis

Today we will talk about pulmonary fibrosis. A concept that has been heard a lot in the media, especially in recent post-pandemic years. The information is extensive, which could create confusion and uncertainty among the general population and patients who already suffer from this group of diseases.
We will start by defining fibrosis. The Association of Spanish Language Academies defines it as the pathological formation of fibrous tissue. Therefore, when talking about pulmonary fibrosis we would be referring to the formation of pathological fibrous tissue that replaces the lung parenchyma. The formation of fibrotic tissue can be the result of healing from a previous inflammatory process, very frequently caused by pneumonia of different etiologies or causes. An example of this is pulmonary tuberculosis, a disease with a high incidence in Mexico, which in its active stage is characterized by causing cough, fever, evening sweats and weight loss. When performing imaging studies we can observe pneumonia and cavitations. Cavitations are necrotic tissue in the lung that ends up forming “pits.” With the correct treatment, these lesions improve and tend to heal, generating fibrotic lesions.
 
Pulmonary cavitation due to pulmonary tuberculosis
 
 

Causes

In addition to this, there is a very heterogeneous group of lung disorders in a region that we call “interstitium” and that are capable of causing fibrosis. We know these as Diffuse Interstitial Lung Diseases (DILD) and they can cause very different clinical and histological data.

ILDs can be caused by exposure to a very wide variety of organic and inorganic agents, occupational or within the home, hereditary or environmental, smoking, medications or autoimmune diseases, such as systemic lupus erythematosus, polymyositis, scleroderma, Sjögren's syndrome, rheumatoid arthritis among many others.

It is generally accepted that there are over 200 different types of inflammatory diseases that will lead to fibrosis of the alveolus, airways and interstitium.

On the other hand, idiopathic pulmonary fibrosis, or in medical jargon known only by the acronym 'IPF', is a very specific type of chronic interstitial fibrosis that occurs idiopathically; that is, spontaneously. IPF is related to a tomographic and histological pattern that we call: “usual pattern”.

IPF is characterized by the absence of inflammation, it is associated with smoking and lung aging; where the lung tissue fibroses spontaneously and progressively. It is considered one of the types of DILD with the worst prognosis that exists today.

COVID-19 AND FIBROSIS

COVID-19, a disease caused by a new coronavirus called SARS-COV2, is characterized by the presence of respiratory symptoms, accompanied in some more severe cases, by the affection of multiple organs and systems of the human body. After exposure to the virus, some people will remain asymptomatic, others will develop mild symptoms limited to the upper respiratory tract. In some cases, the disease will be self-limiting, but others will develop pneumonia that may be mild and go unnoticed or progress to a more serious state with oxygen requirements and even the need for intensive care. Once this inflammatory process is resolved, scarring lesions may develop at the lung level. It is still too early to be sure about the natural history of post-COVID pulmonary fibrosis, but it is generally accepted that most patients will progress to improvement, others will remain static, and a less common group will have fibrosis progression over time. .
 
Natural history of post-covid fibrosis

Lung India 2021 Mar, 38 (Suppl 1):S41-S47

 

CLINICAL PICTURE

The symptoms in ILD are insidious and are characterized by the presence of dry cough and dyspnea (shortness of breath) that will be progressive. When the doctor examines these patients, he generally finds sounds in their lungs that we call “Velcro rattles,” which are very fine noises and denote the presence of fibrotic tissue. Another very common clinical finding is the presence of clubbing or clubbing of the fingers. The rest of the signs and symptoms will depend on the underlying disease or the cause that led to this pathology.

 
 
Clubbing or clubbing
 
 
 

FORECAST

The prognosis will vary with the type of fibrosis that is diagnosed, since in IPF the average survival is 3 to 5 years, while in other types of ILD the disease can be less aggressive with appropriate management.

 

DRIVING

When we suspect a fibrotic disease, it is essential to perform a high-resolution lung tomography (HRCT), in some cases lung biopsy will be required either by freezing (lung cryobiopsy) or taken by surgery.

 
Pulmonary HRCT with honeycombing (fibrosis)
When it comes to inflammatory ILD, it is possible to give corticosteroids or other immunosuppressive medications that can be combined with anti-fibrotic drugs.

Regarding anti-fibrotic treatment, there are only two drugs that have currently shown efficacy in progressive fibrosis: nintedanib and pirfenidone. These medications can be used in IPF and in some types of progressive fibrosis other than IPF, however, studies in post-covid patients are still underway and there are no results that support their use in this group of patients at present.

 

CONCLUSIONS

In conclusion, the term “pulmonary fibrosis” is very ambiguous if we consider that there are a large number of insults that can cause it, in addition to the fact that the clinical range will depend on whether there is an underlying disease. Tomographic characterization is essential to try to classify it; in some cases, studies such as bronchoscopy or lung biopsy will be required. Finally, treatment may consist of the use of corticosteroids, other immunosuppressants, anti-fibrotics and/or in some selected cases, monitoring.

I am Dr. Cecilio Omar Ceballos Zúñiga, specialist in pulmonology and internal medicine [National Institute of Respiratory Diseases (UNAM) and General Hospital of the State of Sonora (UNAM)] and basic training at the Mexicali School of Medicine (UABC) . Co-founder of Breathbaja.

Ced. Prof. 4829126, reg. esp. 6119468 / 7440242

 
We are members of:
  • American Academy of Sleep Medicine
  • Mexican Academy of Sleep Medicine
  • Mexican Society of Pulmonology and Thorax Surgery
  • European Respiratory Society
  • American Thoracic Society
  • Latin American Thorax Association
  • Latin American Society of Respiratory Physiology
  • Mexican Society of Internal Medicine. Mexicali Chapter

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