Are you having these problems with your lungs? Let's talk about Pulmonary Sarcoidosis

Do you sometimes feel short of breath, or have a persistent cough that lasts for days? Maybe you feel tired for no reason, along with chest pain. These can be more than just normal fatigue. Today we are going to talk about a condition that you may not have heard of, but is very important to know about. That is pulmonary sarcoidosis.

What is Pulmonary Sarcoidosis?

Simply put, sarcoidosis is a disease that causes inflammation , or swelling, in various parts of our body. What happens in this is that the cells of our immune system gather and form small lumps. We medically call these lumps granulomas . To be precise, these can be as small as a grain of rice or a little larger.

Now, Pulmonary Sarcoidosis is when this condition specifically affects our lungs . But it is not limited to the lungs. Sometimes it can affect the skin, eyes, lymph nodes, heart, or any other organ in the body. Most of the time, this pulmonary sarcoidosis condition gets better on its own. But in some unfortunate cases, this ongoing inflammation can cause permanent scarring of the lungs. That is why it is so important for all of us to be aware of this condition.

Is this cancer?

Many people, when they hear the name "sarcoidosis", especially when they hear the "lumps" that form, may think, "Oh, I don't know if this is cancer." No, to be clear, sarcoidosis is not cancer. It is a completely different condition. However, some medical studies have found that people with sarcoidosis may have a slightly higher risk of developing certain types of cancer in the future than others. But you should keep in mind that these two diseases are not the same.

What are the symptoms of Pulmonary Sarcoidosis?

Remember, not everyone has this condition, but they don't show any symptoms. Some people can have it without any symptoms. However, if you keep experiencing these things, you should be a little concerned:

• You may experience chest pain . It may feel like a tightness in the middle of your chest, or it may hurt when you breathe.
• You may have a persistent dry cough that doesn't subside no matter how much cough medicine you take.
• You may feel fatigue , tiredness, a general feeling of being unwell, or malaise . It's like having a fever, and your body feels heavy.
• Difficulty breathing (dyspnea) , which means feeling short of breath and having difficulty breathing. You may feel faint even after walking a short distance.
• Sometimes you may hear a whistling sound from your chest when you breathe .

In addition to these things, many people with pulmonary sarcoidosis struggle with depression . It's no wonder that when a chronic illness like this interferes with daily life, work, and social interactions, it can be stressful.

What is the reason for this?

In fact, doctors and scientists still don't know the exact cause of pulmonary sarcoidosis. It's a bit complicated. In some cases, it runs in families, meaning there may be a genetic link . In other cases, it's thought to be caused by environmental factors, such as certain bacteria, viruses, dust, or long-term exposure to chemicals . It can also be a side effect of certain medications . Researchers are still studying this to find out exactly what happens.

How common is this condition? Who is more likely to get it?

Although there are no exact statistics on this in Sri Lanka, when we look at other countries of the world, for example in countries like the United States of America, it is reported that about one and a half to two hundred thousand people suffer from this condition of sarcoidosis. On average, while about 11 people out of every 100,000 white people develop this condition, in populations of African descent this number increases to about 34 out of every 100,000. In general, there is about a 2.4 percent chance of someone developing this condition.

Sarcoidosis often first affects the lungs.

Pulmonary Sarcoidosis can affect anyone, regardless of gender, age, or race. However, the following people have been found to be at a slightly higher risk:

• People of African or Northern European (e.g. countries like Denmark, Norway, Sweden) origin.
• People who work in environments with pesticides, mold, dust, or other substances that can cause inflammation.
• People who take certain medications long-term. For example, some medications for HIV infection, anti-cancer drugs, or medications that make the immune system overactive.
• If someone in your family has had pulmonary sarcoidosis before, that means there may be a genetic influence.
• Women are also more prone to this condition than men.

How do you recognize this?

If your doctor suspects pulmonary sarcoidosis based on your symptoms or other tests, he or she may order several tests, such as:

• Chest X-ray: This is the first major test done. It can check for abnormalities in the lungs and swollen lymph nodes.
• CT scan: A CT scan can produce clearer, cross-sectional images than an X-ray. This helps to see the condition of the lungs in more detail.
• Pulmonary function testing: This involves having you blow into a machine to measure how well your lungs are working, how much air you can take in and out, and how well you can absorb oxygen into your blood (e.g., gas diffusion test) .
• Biopsy: This is the best way to confirm the diagnosis. This involves taking a small piece of tissue from the lungs or a swollen lymph node and examining it under a microscope to see if there are any granulomas. This tissue sample can be obtained through a procedure called bronchoscopy (a thin tube with a camera inserted into the airways).
• Blood tests: Check for things like inflammation levels, calcium levels, kidney and liver function, etc. Sometimes the level of angiotensin-converting enzyme (ACE) is checked, as this can be elevated in sarcoidosis.
• If necessary, other specialized scans such as MRI or positron emission tomography (PET) scans may also be performed.

What are the stages of Pulmonary Sarcoidosis?

Based on your test results, especially what you see on your chest X-ray, and your symptoms, your doctor may assign a stage to the disease. These stages range from asymptomatic to severe lung damage:

• Stage 0: Chest X-ray is completely normal. There are no signs of granulomas. Symptoms may also be absent.
• Stage I: The hilar lymph nodes on either side of the chest are swollen, but there is no visible damage to the lung tissue. In most cases, symptoms may be absent.
• Stage II: Lymph nodes are swollen, and there are signs of granulomas in the lung tissue. These people may develop symptoms.
• Stage III: Granulomas have spread throughout the lung tissue, but swelling in the lymph nodes may be reduced or absent.
• Stage IV: This is the most severe stage. Pulmonary sarcoidosis has caused permanent, irreversible scarring of the lung tissue, known as pulmonary fibrosis. This causes the lungs to become very weak.

Remember, these stages do not always directly correlate with the severity of the disease. Some people may experience severe symptoms even in stage I.

What are the treatments for this?

There is good news! Not everyone with pulmonary sarcoidosis will need treatment. In most cases, especially in stage I, the condition goes into spontaneous remission without any treatment . Your doctor will just monitor you for a while.

However, if your symptoms are severe, if your lung function is affected, or if it affects a vital organ like your eyes or heart, treatment is needed.

• The main medication given is prednisone . This is a type of corticosteroid . It is used to control inflammation in the body and the overactivity of the immune system. However, because this medication can cause side effects such as weight gain, an increased risk of diabetes, and thinning bones when used for a long time, the doctor will give it in the lowest possible dose and for the shortest possible time.
• For people who have difficulty tolerating prednisone, or who need long-term treatment, doctors may recommend other medications such as:
• Medicines that suppress the immune system, such as methotrexate and azathioprine .
• Tumor necrosis factor (TNF) inhibitors, such as infliximab or adalimumab . These are often used for conditions such as rheumatoid arthritis.
• Sometimes antimalarial drugs like hydroxychloroquine are used to treat skin and joint symptoms.

Very rarely, in severe cases (stage IV) where permanent lung damage has occurred and breathing difficulties are severe, the following may be necessary:

• Pulmonary rehabilitation: Special exercise and counseling programs.
• Supplemental oxygen .
• Finally, a lung transplant surgery .

Can this be prevented?

Unfortunately, scientists still don't fully understand what causes pulmonary sarcoidosis, so there's no proven way to prevent it. However, some cases have been linked to exposure to environmental factors like pesticides, dust, and mold, so staying away from those things as much as possible and living in an environment with clean air can help keep your lungs healthy. It's also important to quit smoking altogether.

Is this life-threatening? Can it happen again?

Most cases of pulmonary sarcoidosis, especially if the symptoms are mild, go into spontaneous remission . Other cases resolve completely with short-term treatment. Such cases are not life-threatening.

However, if this condition persists for a long time and does not respond well to treatment, permanent scarring (fibrosis) of the lungs can occur. If this happens, it can reduce lung function, increase breathing difficulties, and sometimes shorten a person's life expectancy.

Some people can achieve a permanent remission, meaning the disease is completely gone and does not come back.

However, pulmonary sarcoidosis can recur even after remission or successful treatment . This is sometimes called a 'flare-up'. Therefore, even if the disease is improving, it is important to continue to go for tests as prescribed by your doctor.

What are the possible complications of Pulmonary Sarcoidosis?

If those granulomas we talked about earlier don't heal properly and persist, they can cause complications like:

• Pulmonary fibrosis: This is the most serious and dangerous complication. The lung tissue becomes scarred and the lungs become stiff. This makes it difficult to breathe and causes permanent damage to the lungs.
• Bronchiectasis: This is a condition in which the air passages (bronchi) in the airways become permanently widened and damaged. This can lead to mucus buildup and frequent lung infections.
• Pulmonary hypertension: This is high blood pressure in the arteries that carry blood to the lungs. This can also affect the heart.
• Eye problems: If sarcoidosis affects the eyes, conditions such as uveitis can occur, leading to vision loss and even blindness.
• Heart disease: Rarely, it can affect the heart, causing conditions such as irregular heartbeat and heart failure.
• Effects on the kidneys: Increased calcium levels in the blood can lead to the formation of kidney stones and impaired kidney function.
• End-stage lung disease: This is the final stage of lung disease. In this, your lungs are no longer able to remove carbon dioxide from your body or provide enough oxygen to your body.

How do I take care of myself as someone with Pulmonary Sarcoidosis?

If you have been diagnosed with Pulmonary Sarcoidosis, don't panic. These tips can help you manage the condition, recover, and live a healthy life:

• Stay away from things that are harmful to your lungs. Protect yourself from things like dust, chemicals, paint fumes, pesticides, and mold. Don't smoke at all. Don't be in places where other people smoke (secondhand smoke).
• Eat a nutritious, balanced diet. Eat more vegetables, fruits, clean fish, and whole grains. Reduce processed foods, sugar, and foods high in fat.
• Exercise regularly. Ask your doctor what exercises are right for you and do them. Things like walking and swimming are good.
• Maintain a healthy weight. Being overweight can increase your risk of breathing problems.
• Be sure to attend all scheduled tests and appointments with your doctor on time. Don't skip blood tests, lung function tests, or X-rays.
• Take all prescribed medications exactly as your doctor tells you, at the time and for the duration prescribed. Do not stop taking the medication or change the dosage on your own.
• Take care of your mental health. Living with a disease like this can be stressful. Talk to your doctor, family, and friends about your feelings. Seek psychiatric help if necessary.
• If you experience any new symptoms or if your symptoms seem to be getting worse, especially changes in breathing patterns, chest pain, or a severe cough, tell your doctor right away.

When should I seek medical advice?

Your doctor will tell you what symptoms you should be especially aware of and what to do if they occur. These symptoms can vary depending on where the sarcoidosis is located. If you have pulmonary sarcoidosis, you should seek medical attention immediately if you have a sudden change in your breathing, severe chest pain, shortness of breath, or bloody phlegm. Also, see your doctor if you have a fever, body aches, or a persistent cough that lasts for several days.

The most important things you need to remember (Take-Home Message)

Okay, so the pulmonary sarcoidosis we talked about today is a disease of unknown cause that causes inflammation and small lumps called granulomas in the lungs. For most people, it resolves on its own, or gets better with short-term treatment. So don't be unnecessarily afraid or worried about this.

The most important thing is that if you have symptoms like a persistent cough, shortness of breath, or chest pain, don't just ignore it and see a doctor for a checkup. Early detection and proper treatment are very important in these types of illnesses. Following your doctor's instructions, taking your medications correctly, and going for regular checkups will help you a lot. Don't be afraid to talk to your doctor or family about any questions or concerns you may have. Stay positive, stay healthy!

` Pulmonary Sarcoidosis, Sarcoidosis, Granulomas, Lung Disease, Respiratory Distress, Cough, Inflammation, Pulmonary Fibrosis