Sarcoidosis - Causes, Symptoms, Diagnosis, and Treatment

What Is Sarcoidosis?

Sarcoidosis is characterized by the growth of clumps of inflammatory cells (granuloma), commonly affecting the lungs and lymph nodes. The other organs that can be affected are the skin, eyes, and heart. It is believed to be caused by the body's immunity responding to some unknown substance, such as microorganisms, dust, chemicals, and self-proteins (the body's proteins). As of now, there is no cure for this disease, but some cases get better without any medical treatment. Treatment is required when these granulomas damage organs.

What Are the Various Stages of Pulmonary Sarcoidosis?

According to the Siltzbach classification system, pulmonary (lung) sarcoidosis is categorized into various stages, which include:

• Stage 0: No evidence of pulmonary sarcoidosis on X-rays. Lungs and lymph nodes appear normal.

• Stage 1: Presence of granulomas in lymph nodes only.

• Stage 2: Presence of granulomas in both lymph nodes and lungs.

• Stage 3: Presence of granulomas in lungs only.

• Stage 4: X-rays reveal pulmonary fibrosis, indicating permanent scarring of the lungs.

What Symptoms Does Sarcoidosis Result In?

The signs and symptoms vary depending on which organ is affected by the formation of granulomas.

Common Symptoms:

• Tiredness or fatigue.

• Lymph node enlargement.

• Weight loss.

• Tenderness and inflammation of joints.

The symptoms when granuloma is present in the lungs are:

• Chronic dry cough.

• Shortness of breath or dyspnea.

• Chest pain.

• Wheezing.

The symptoms when this disease affects the skin are:

• Red or purplish skin rashes on the ankles.

• Large lesions on the nose and cheeks.

• Nodule-like skin growth forms under the skin.

• Some areas of the skin become darker or lighter.

The symptoms when granuloma is present in the lungs are:

• Chest pain.

• Dyspnea.

• Syncope.

• Edema.

• Heart arrhythmias.

• Heart palpitations.

The symptoms seen when the eye is affected are:

• Blurry vision.

• Eye pain.

• Eyes become dry and itchy.

• Eye redness.

• Light sensitivity.

Sometimes, sarcoidosis progresses gradually, and the symptoms last for years, while some cases result in sudden symptoms that disappear independently. Sometimes, sarcoidosis is diagnosed during a routine chest X-ray for patients with no symptoms. This disease can also affect the nervous system, liver, spleen, calcium metabolism, and bones.

What Can Cause Sarcoidosis?

Sarcoidosis is believed to be affected by genetics, but the exact cause is not known. People who have a genetic predisposition develop this disease when triggered by infections caused by microorganisms, chemicals, or dust.

These triggers make the immune system overreactive, resulting in the collection of inflammatory and immune cells called granulomas. Granulomas can build up in any organ and affect its functioning.

What Factors Increase the Risk of Developing Sarcoidosis?

The risk factors for sarcoidosis are:

• Women are more prone.

• Africans, Northern Europeans, and African Americans are more susceptible.

• A positive family history.

• Age: Sarcoidosis can develop at any age, but the risk increases with age, particularly after 55.

• Environment: Exposure to insecticides, mold, or other inflammatory substances in the living or working environment can elevate one’s risk. Occupations such as healthcare, the automotive industry, farming, or firefighting may involve exposure to these substances.

• Medications: Some HIV medications and monoclonal antibodies employed in cancer therapy or for regulating an overactive immune system may also increase the risk of sarcoidosis

• Other Medical Conditions: Sarcoidosis can also arise from other medical conditions, like lymphoma, a form of blood cancer.

When to Consult a Doctor?

If an individual experiences the following symptoms, it is best to consult a doctor immediately:

• If breathing becomes labored.

• If the heart is beating too fast.

• If one has problems with vision.

• If eyes are paining.

• If one develops facial numbness.

• If one becomes sensitive to light.

How Does a Doctor Diagnose Sarcoidosis?

Sarcoidosis is difficult to diagnose in its early stages, as it usually causes no symptoms. Because the symptoms are not specific to this disease, it is often misdiagnosed.

The doctor will first perform a physical examination, listen to heart and lung sounds, and check if the lymph nodes are enlarged. To rule out all possible causes of symptoms, the doctor might suggest the following tests:

• Blood and Urine Tests - Kidney and liver function tests check how well your organs are functioning.

• Imaging Tests (Chest X-ray, CT scan) - To see how your heart and lungs are.

• Lung Function Tests - To check how efficiently your lungs are working.

• Electrocardiogram (ECG) - To monitor your heart.

• Eye Examination - To check your vision.

• Magnetic Resonance Imaging (MRI) - To see if there are granulomas in the heart, brain, or spinal cord.

• Biopsies - If any imaging test shows lesions or granulomas or if you have skin lesions, then the doctor might take a biopsy from the affected areas to check for cells in the granulomas.

What Are the Treatment Options for Sarcoidosis?

There is no known cure for sarcoidosis, but the symptoms go away in some patients without any treatment. If you are experiencing symptoms, your doctor will prescribe medicines to relieve those symptoms and help the affected organ function properly. The treatment options include:

1. Medications:

• Corticosteroids - This helps in reducing inflammation.

• Immunosuppressants - Medicines like Methotrexate and Azathioprine are used to suppress the immune system's action.

• Hydroxychloroquine - To treat high blood calcium levels.

• Tumor necrosis factor-alpha (TNF-alpha) inhibitors - The drugs of this group are prescribed when no other treatment option has worked.

2. Other Treatments:

• Physiotherapy - To improve muscle strength.

• Pulmonary rehabilitation - To relieve breathing problems.

• Cardiac pacemaker - To correct arrhythmias.

• Organ transplantation - It is only done if the organ, such as the lungs, heart, or liver, is severely damaged.

3. Lifestyle Changes:

• Go for regular follow-ups.

• Never skip the medicines or stop them without consulting the doctor.

• Eat healthily.

• Maintain a healthy weight.

• Reduce stress.

• Exercise regularly.

• Sleep at least seven hours every day.

What Are the Complications of Sarcoidosis?

In some patients, sarcoidosis can result in the following chronic complications:

1. Pulmonary Fibrosis - If left untreated, sarcoidosis of the lungs can cause permanent scarring or pulmonary fibrosis. This can result in breathing problems and pulmonary hypertension.

2. Eye Problems - Sarcoidosis can affect the eye and damage the retina, causing blindness. It can also result in cataracts and glaucoma.

3. Heart Problems - Sarcoidosis of the heart can cause arrhythmias and abnormal heart function.

4. Kidney Diseases - As sarcoidosis affects calcium metabolism, it can cause kidney stones and affect kidney function.

5. Central Nervous System Problems - When granuloma affects the brain or spinal cord, it can cause permanent nerve damage and paralysis.

6. Infertility.

What Precautions Should Be Taken While Living With Sarcoidosis?

• Focus on a nutritious diet rich in lean meats, proteins, fruits, vegetables, whole grains, and limited sugar intake. Maintain a healthy weight.

• Since some sarcoidosis medications can strain the liver, it is best to avoid alcohol to minimize the risk of liver damage.

• Smoking can exacerbate lung symptoms, so quitting smoking is highly recommended. Seek assistance from a smoking cessation program if needed.

• Regular physical activity can enhance mood, strengthen muscles, and alleviate fatigue associated with sarcoidosis.

• Employ stress-reduction techniques such as meditation to cope with the challenges of a chronic illness like sarcoidosis.

• Prioritize good sleep hygiene to reduce inflammation and enhance overall well-being. Consider meeting with a counselor or joining a support group if someone is struggling emotionally or mentally with sarcoidosis.

What Is the Prognosis for Sarcoidosis?

For many cases, it is temporary and can clear up with or without treatment, while for others, it may become a long-term condition causing lasting harm. Around two-thirds of people with sarcoidosis recover within two to three years, especially those with Lofgren syndrome, typically resolving within six months to two years. However, sarcoidosis may persist for more than three years, leading to chronic illness. Only a small percentage will experience permanent organ damage, with lung scarring being the most common complication.

Conclusion

If someone has been diagnosed with sarcoidosis, it is important to consult a doctor regularly, even if symptoms are getting better. Even if the doctor advises no medical treatment, one must monitor the condition with regular tests. With proper treatment, most patients lead an almost active and healthy life. The symptoms take about a couple of years to improve, with or without treatment. But in rare cases, this condition can become chronic. For any doubts about sarcoidosis, consult a doctor now.