Overview of sarcoidosis

Sarcoidosis, an inflammatory condition, is a disease that causes small aggregations of cells, called granulomas, to develop in various body parts. A granuloma is a kind of inflammation induced by the immune system. It is constituted of a collection of white blood cells that protects the body from anything the immune system considers toxic. Granulomas have a hard, lumpy texture because of the dense surrounding tissue and can alter how the affected organs work.

While the origin of sarcoidosis is unknown, an abnormal immune response may be the cause. Many related illnesses, like rheumatoid arthritis and lupus, develop when the immune system attacks the body and are known as autoimmune diseases. All ethnicities are susceptible to developing sarcoidosis at any age. However, it is most common among African Americans, Scandinavians, and adults aged 30 to 50. It can affect any body region, with the risk varying according to race, gender, and age.

Sarcoidosis symptoms can vary greatly. However, some of the most frequent include: 

• Fatigue: it refers to feeling exceedingly exhausted or lacking energy
• Breathlessness: trouble catching one's breath, particularly during intense activity
• Persistent cough: it does not go away, may be dry or produce sputum
• Joint pain: it refers to soreness, swelling, or stiffness in the joints
• Skin rashes: red or purple areas on the skin that may be elevated and itchy
• Eye problems: including impaired vision, eye discomfort, and light sensitivity
• Enlarged lymph nodes: swollen or sensitive lymph nodes, often in the neck, armpits, or groin
• Fever: continuous low-grade fever

Diagnosis of sarcoidosis

If a physician suspects a patient is suffering from sarcoidosis, several analyses are done according to the symptoms. Imaging tests such as chest X-rays, CT scans and MRI, and biopsies from the organ affected (to visualise the granulomas) are the most common. They are taken from the skin lesions, or the affected lung or lymph nodes(if affected).

Other tests include:

• Pulmonary function test: it is a non-invasive test that measures the function of the lung
• Electrocardiogram (ECG): it measures the electricity of the heart
• Slit lamp examination: which determines several eye problems 
• Lab tests: routine tests to evaluate  how well organs function

Understanding treatment options

Early treatment of sarcoidosis can prevent scarring of the affected organ, which loses its function, and relieve the patient’s symptoms. Treatment options include medications, lifestyle modifications and surgery.

Medications

The severity of the symptoms and the level of organ damage influence the treatment strategy. Moreover, patients may recover without getting pharmacological treatment.² In the UK, 42% of patients in a study about sarcoidosis did not receive this type of treatment.³

New treatments may bring new side effects, thus reporting any changes during the treatment is recommended. For example, when pulmonary (lung) sarcoidosis turns into pulmonary fibrosis, treatment should be updated to include medication for the lungs, rehabilitation, oxygen therapy and doctors may suggest a lung transplant in severe cases.⁴  

Corticosteroids

Corticosteroids are the main treatment for remission of pulmonary sarcoidosis with impaired lung function, cardiac sarcoidosis, neurosarcoidosis, sarcoidosis of the eyes, and affected skin. They delay fibrosis by decreasing the inflammatory process, leading to better symptoms and delay of disease progression. Nevertheless, the treatment is not curative and relapses are frequent when tapered.  Physicians usually weigh the pros and cons of using corticosteroids in sarcoidosis because of their side effects.⁵ Osteoporosis, gastritis, steroid-induced diabetes and weight gain could have more harmful effects on the patients than sarcoidosis itself, especially in long treatments.⁶ 

Disease-modifying antirheumatic drugs (DMARDs)

In pulmonary sarcoidosis, when patients suffer a relapse, additional treatment with disease-modifying antirheumatic medications (DMARDs) would be recommended to reduce the dose of corticosteroids and avoid their toxicity.⁷ Examples of these are:

• Methotrexate and azathioprine

They suppress the immunity system, decreasing the process of damage. Originally used to treat rheumatoid arthritis.  Methotrexate is commonly used in pulmonary sarcoidosis.

• Hydroxychloroquine 

It is an antimalarial medication that can be used to treat sarcoidosis. Side effects may be stomachache and eye problems

Biological agents

If you don’t respond to corticosteroid or DMARD treatments, doctors will prescribe you a biological modifier. The most used is infliximab, an antibody that impedes the function of anti-tumour necrosis factor-alpha (TNF-alpha) (a messenger in the immune response).⁷ 

Lifestyle options 

Incorporating and changing some lifestyle habits can help reduce the risk of sarcoidosis and decrease inflammation.

• Smoking cessation to avoid increasing lung damage 
• Low alcohol consumption to decrease inflammation
• A well-balanced diet, anti-inflammatory diet is a good example
• Weight reduction, as obesity increases the risk of sarcoidosis
• Stress reduction and mental health improvement
• Regular exercising improves respiration, shortness of breath and muscle weakness
• Sleep adequately from 7 to 9 hours per day to avoid tiredness

Surgery

You may need to consider an organ transplant when the lungs, heart, or liver are seriously affected. Oxygen therapy is required in cases with severe lung damage, using a nasal mask and a tank.

Monitoring the progression of the disease

Numerous biomarkers, imaging methods, and multidisciplinary evaluations have been developed to monitor sarcoidosis and ensure it is managed effectively. When it becomes a chronic disease, follow-up care might be a lifetime. Your doctor will monitor your symptoms, assess the efficacy of therapies, and look for side effects. Regular testing based on your condition may be part of the monitoring process: chest X-rays every 6 to 12 months, lab and urine testing, ECGs, slit lamp examination and examinations of the lungs, skin, and any other affected organs.

Remission and relapse

Remission means the disappearance of symptoms while relapse means the reappearance of symptoms or lesions when medications are tapered or up to 1 year after stopping the treatment. In 2-3 years, about two-thirds of the patients will be in remission and the rest will develop a chronic disease. If your sarcoidosis goes into remission, your doctor may decide to gradually discontinue your treatments, especially corticosteroids. Mostrelapses, or flares, occur during the first 6 months of discontinuing medication, therefore it is critical to monitor your health and continue to see your healthcare practitioner.^10,11 The longer you remain symptom-free, the less likely you are to relapse.

Summary

Sarcoidosis is a disease characterised by small lumps of cells, called granulomas, formed in various body parts, that alter the function of the affected organs. The cause of sarcoidosis is not fully understood but it may result from an abnormal immune response. The disease can affect people of all races and ages, with common symptoms such as fatigue, breathlessness, cough, joint pain, skin rashes, eye problems, enlarged lymph nodes, and fever. 

Diagnosis involves various imaging tests, biopsies, pulmonary function tests, ECG, slit lamp examination, and routine lab tests. Early treatment is beneficial to prevent scarring of affected organs and relieve symptoms. Treatment options include medications, lifestyle modifications, and, rarely, surgery. Medications, such as corticosteroids and disease-modifying antirheumatic drugs, manage symptoms and prevent organ damage. Lifestyle modifications include smoking cessation, balanced diet, weight reduction, stress reduction, and regular exercise. Regular monitoring and follow-up care are necessary as sarcoidosis may go into remission or relapse over time.