Pulmonary Fibrosis vs Cystic Fibrosis – Symptoms, Causes, Treatment, Prevention

Pulmonary Fibrosis (PF)

It is a respiratory disease that occurs when lung tissue becomes scarred or damaged. This can prevent the body from getting sufficient oxygen and eventually can lead to heart failure, respiratory failure, or other complications.

As the scarring progresses, it may become harder to breathe, therefore, some patients may require oxygen to help them breathe.

Approximately 5 million individuals worldwide are affected by PF. Nevertheless, the actual numbers may be considerably higher due to misdiagnosis.


Common symptoms of PF may include:

  • aching joints and muscles;
  • fatigue;
  • rounding and widening of the toes or tips of the fingers;
  • unexplained weight loss;
  • a dry cough;
  • shortness of breath.


It can be caused by a variety of conditions including:

  • being exposed to allergens, like – mold, bird feathers, or asbestos;
  • infections;
  • certain drugs – amiodarone, nitrofurantoin, cyclophosphamide methotrexate, or bleomycin;
  • chronic conditions, such as – rheumatoid arthritis or lupus;
  • exposure to ionizing radiation (energy in the form of particles), especially radiation therapy that is used to treat tumors of the chest;
  • chronic inflammatory processes, including – Wegener’s granulomatosis (a systemic condition that involves both polyangiitis and granulomatosis) or sarcoidosis.

In most cases, healthcare professionals do not know precisely what causes PF and when no known cause is identified, it is called idiopathic pulmonary fibrosis (IPF). When PF is idiopathic, it most frequently occurs in patients 50 years of age and older, however, individuals of any age can develop this condition.

Moreover, approximately 15% percent of people with IPF have another family member afflicted by the condition.


There is no cure for PF, however, new therapies can slow the worsening of the condition in some sufferers. Some of these medical procedures include:

  • pulmonary rehabilitation, also referred ad pulmonary rehab, is a program of exercises and advice to help the patient to breathe more easily;
  • breathing oxygen through a mask – this can be done while you’re out and/or while you are at home;
  • drugs – there are two medicines approved to treat IPF: Esbriet (pirfenidone) and Ofev (apnintedanib)
  • for some patients, a lung transplant may be the best treatment option when other treatments have not worked.


Prevention methods include:

  • quitting smoking and avoiding second-hand smoke are good prevention methods not only for PF but also for many other respiratory conditions (like – pulmonary embolismreactive airway disease, or pneumonia).
  • it is estimated that hands spread 75% of common infectious respiratory diseases. Therefore, washing hands with water and soap can prevent this type of infection.
  • keep your bathrooms and sinks free from mildew or mold. Keep your house free from excess dust and clean.
  • swimming, walking, running or any type of physical activity will help keep the lungs healthy.
  • good nutrition is vital to help the body to resist any kind of infections. For respiratory problems, it is recommended to eat anti-inflammatory foods.

Cystic Fibrosis (CF)

It is a progressive, genetic disease that affects the secretory glands, that are responsible for producing sweat and mucus. This can lead to severe problems, like – respiratory failure, infections, and malnutrition. CF usually affects the lungs, liver, pancreas, sinuses, intestines, and sex organs.

This disease can be life-threatening, and people with CF tend to have a shorter-than-normal lifespan.


Common symptoms include:

  • slow growth;
  • trouble breathing;
  • a nagging cough;
  • recurrent sinus infections;
  • amenorrhea (the absence of menstruation);
  • delayed secondary sexual development;
  • undescended hydrocele or testicles;
  • recurrent wheezing;
  • oily stools;
  • loose stools;
  • inability to gain weight despite taking in enough calories and having a good appetite.


It is caused by a faulty gene which a child inherits from both their parents. This is called the CFTR gene or “cystic fibrosis transmembrane conductance regulator.”

If children inherit only one copy of CFTR gene, they won’t develop CF. However, he will be a “carrier” of this condition. This actually means there’s a chance he could pass CFTR gene on to his child.

To have this condition, a child needs to inherit one copy of the CFTR gene from each parent. If both parents have CFTR gene, there is a 25 percent chance the child will have this condition.

Most children with this condition are diagnosed by age 2. For a small number of patients, CF is not detected until age 18 or older. These patients usually have a milder form of CF.  In addition, sufferers with this condition have a higher chance of developing osteoporosis (a condition in which bones fracture more easily) or type 2 diabetes mellitus.


There is no known cure for CF. Lung infections are usually treated with antibiotics that may be inhaled, given intravenously, or by mouth. Also, the patient may receive drugs to make the sticky mucus in the lungs thinner, like – hypertonic saline, dornase alfa, and mannitol dry powder.


CF is a genetic disorder that cannot be prevented.

Pulmonary Fibrosis vs Cystic Fibrosis – Differences

Pulmonary fibrosis is a condition characterized by scarring in the lungs that can be prevented by lifestyle changes.

On the other hand, cystic fibrosis is a hereditary disease that affects the lungs and digestive system. CF is one of the most frequent inherited diseases among Caucasians and cannot be prevented.

Image credit – Shutterstock

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