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Pulmonary Alveolar Proteinosis - Symptoms, Causes, Risk Factors, Diagnosis, and Treatment

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Pulmonary Alveolar Proteinosis - Symptoms, Causes, Risk Factors, Diagnosis, and Treatment

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Pulmonary alveolar proteinosis (PAP) is a medical condition affecting the lungs and can be fatal if left untreated. This article explains this condition in detail.

Medically reviewed by

Dr. Jebin Abraham

Published At June 18, 2021
Reviewed AtMay 6, 2024

Introduction

Pulmonary alveolar proteinosis (PAP) is a rare condition that affects the lungs. It is caused by the buildup of surfactants in the lungs, which leads to the blockage of air sacs or alveoli.

What Is a Surfactant?

A surfactant is a naturally occurring substance that decreases the surface tension in the lungs and helps us breathe. There are three types of pulmonary alveolar proteinosis: congenital, acquired, and secondary. This condition can lead to mild to moderate breathing issues, and if treatment is not given, it can even lead to death.

What Are the Signs and Symptoms?

This condition can be symptomatic or asymptomatic. When a patient develops symptoms, the following can be experienced by the patient:

  • Breathing difficulty - It is the most commonly reported symptom.

  • Coughing - This can sometimes be associated with blood or mucus.

  • The face can be discolored and have a bluish color.

  • Loss of weight

  • Tiredness

  • Fever, which can be of low-grade

The above-mentioned symptoms can progress to severe symptoms, which can lead to impairment of the lung and respiratory failure if left untreated. Hence, reporting it to a physician when you suspect or experience any symptoms is important.

What Are the Causes of Pulmonary Alveolar Proteinosis?

As mentioned above, surfactants play a major role. Surfactants are the substances that coat the lungs. Anything in excess can also lead to abnormality. An increase in the levels of surfactants can lead to the development of PAP. An increase in the levels of surfactants can cause blocking of the lungs. Hence, the transportation of oxygen from the lungs to blood is affected. Thus, the symptoms of difficulty breathing happen, but the exact cause has not been established yet.

Are There Any Risk Factors for Pulmonary Alveolar Proteinosis?

The prevalence of this disease, according to the PAP Foundation, is 0.7 people per million. Hence, it is justified as a rare disease. Most cases have been reported to occur in the later stages of life. Most likely, it is known to affect people between the ages of 30 and 50. Males are known to be more affected than females.

The known risk factors or associations with the disease are as follows:

  1. Any immune response of the body that is known to stop or affect the breakdown of surfactants in the body.

  2. Various types of cancer exist.

  3. Any lung infection, including pneumonia.

  4. Getting exposed to toxic particles can happen either professionally or in general. Some of the known risky toxic particles include silica or aluminum dust.

How Can Pulmonary Alveolar Proteinosis Be Diagnosed?

When you visit your physician, you will be asked questions about your signs and symptoms and your personal and medical history. If your doctor suspects this infection in you, you will be asked to undergo the following tests for a confirmatory diagnosis.

  • Chest X-ray - This is used for checking the presence of any white patches in the lungs.

  • Complete Blood Tests - This will help in checking for the presence of any antibodies in the blood that could be related to PAP.

  • Along With a Chest X-ray - a CT scan can be done to check for the presence of white spots in the lungs.

  • Pulmonary Function Tests - This will help the physician understand your lungs' functioning and capacity.

  • A Bronchoscopy Can Be Done With a Saline Wash - This will help in obtaining a fluid sample from the patient’s lungs.

  • Finally, a lung biopsy may be performed to check for the disease.

How Can Pulmonary Alveolar Proteinosis Be Treated?

The treatment depends on the condition's severity and various other factors like patient factors, etc. In mild cases, it is self-limiting and does not require treatment. In the case of certain mild symptoms, oxygen therapy might be required. In case of severe symptoms, the physician will wash the surfactant using a saline solution.

Depending on the affected site, it can be done only in a small part of the lung or the entire lung. When the whole lung needs to be washed, it is called whole lung lavage. In this procedure, the lung is cleansed one by one. When one lung is filled with saline solution and drained, the other lung is touched as it must be ventilated.

The treatment or saline wash can be enough just once or may require multiple times. It depends from person to person. Apart from this, your doctor may prescribe you a blood-stimulating medication. It is a new treatment modality and has shown good results. If nothing works, your doctor may suggest a lung transplant.

How Does It Feel Living With Pulmonary Alveolar Proteinosis?

A patient who has severe PAP requires treatment for sure. About 20% of patients suffering from PAP have a death rate within 5 years of diagnosis. The known causes of death are either respiratory failure or lack of oxygen in the blood.

For the rest of the population, PAP can be managed with treatment. The patient can have a relatively normal life after being diagnosed with PAP and getting treatment. Still, the patient may experience mild difficulty breathing or shortness of breath. Also, patients may develop permanent lung scarring, and thus, their lung capacity may be reduced. However, the occurrence of this condition is rare. All those who get PAP have an increased risk of developing pneumonia, which is a lung infection.

What Is the Life Expectancy of Pulmonary Alveolar Proteinosis?

In cases of severe PAP, life-threatening respiratory failure can occur. The disease can vary from spontaneous remission to stability. A severe infection or respiratory failure can cause death.

Conclusion

Pulmonary Alveolar proteinosis is a medical condition that affects the lungs and can be fatal if left untreated. Hence, it is important to consult a physician as early as possible to improve the prognosis. Online medical platforms have made consulting your physician or a specialist easy by connecting you digitally. Hence, to know more about this condition, consult a specialist online.

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Dr. Jebin Abraham
Dr. Jebin Abraham

Pulmonology (Asthma Doctors)

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