Introduction
Pulmonary alveolar proteinosis (PAP) is a condition that rarely occurs and affects the lungs. The mechanism of this condition is the building up of surfactants in the lungs. This 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 in breathing.
There are three types of pulmonary alveolar proteinosis; they are congenital, acquired, and secondary. This condition can lead to mild to moderate types of breathing issues. If treatment is not done, 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 also if left untreated. Hence, it is important to report to a physician when you suspect or experience any symptom in you.
What Are the Causes of Pulmonary Alveolar Proteinosis?
As mentioned above, surfactants play a major role. Surfactant is the substance that coats 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 in 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. The majority of cases have been reported to occur in the later stages of life. Most likely, it is known to affect people who are between the age of 30 and 50. Males are known to be more affected than females.
The known risk factors or associations of the disease are as follows:
-
Any immune response of the body that is known to stop or affect the breakdown of surfactants in the body.
-
Various types of cancers.
-
Any lung infection, including pneumonia.
-
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, your personal and medical history. Following that, if your doctor suspects this infection in you, you will be asked to undergo the following tests for 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 which 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 to understand the functioning of your lungs and their capacity.
-
A Bronchoscopy Can Be Done With a Saline Wash - This will help in obtaining a fluid sample from the patients’ 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 severity of the condition along with various other factors like patient factors, etc.
In mild cases, it is self-limiting and does not require treatment as such. In the case of certain mild symptoms, it might require oxygen therapy. In case of severe symptoms, washing the surfactant will be done by the physician using a saline solution. It can be done only in a small part of the lung or the entire lung, depending on the affected site. 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 completely being filled with saline solution and drained, the other lung is touched as it has to be kept 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 people, PAP can be managed with treatment. The patient can have a relatively normal life after the diagnosis of PAP and getting treatment. But still, the patient may experience some mild range of difficulty in breathing or shortness of breath. Also, patients may develop permanent scarring in the lungs, and thus, the lung capacity may be reduced. But the occurrence of this condition is rare. All those who get PAP have an increased risk of developing pneumonia, which is a lung infection.
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 both digitally. Hence, to know more about this condition, consult a specialist online.
