Overview of sarcoidosis in children
Sarcoidosis is a rare inflammatory condition in children characterised by the formation of small patches of swollen tissue called granulomas in several body sites.1 The main organs affected by sarcoidosis are the lungs and the lymph nodes.2 However, depending on the person’s skin tone, tender reddish-brown bumps, can also develop on the skin.1
The lymph nodes are known to help the body fight infection. The immune system protects the body from harmful particles, called antigens. In sarcoidosis, the immune system has an exaggerated response to an unknown antigen at the target organ location.3 Immune cells called macrophages increase expression of surface molecules called major histocompatibility complex class (MHC) II, which initiate an inflammatory response by presenting the unknown antigen to other immune cells, known as T helper cells, which multiply and divide to fight the infection.3
The exact cause of sarcoidosis is not currently known,however, researchers believe factors such as chemicals, drugs and genetics may play a role in initiating the disease.3 They also believe that there may be an association between the genetic predisposition of a susceptible person (i.e., having a higher chance of developing a certain condition because of the genes inherited from parents) to an environmental agent that triggers the immune system to overreact, leading to the formation of the granulomas.3
Granulomas impair the normal function of the lung, and they are not limited to this organ; they can also form in other body sites such as the heart or nervous system.2 When this happens, the child may experience symptoms in that part of the body. Additionally, the disease can also affect the liver, spleen and eyes3.
The symptoms typically associated with sarcoidosis include:2
- Fever
- Fatigue
- Changes in weight
- Chest pain
- Skin rash
- Joint pain
Sarcoidosis is usually more common in adults and can sometimes manifest in babies and infants, but it is more likely to affect older children and teenagers.2
Sarcoidosis affects children differently as some may experience symptoms while others won’t. In some cases, the condition can only be detected if the child has a chest X-ray done. Older children have been noted to present with a disease that affects multiple organ systems or tissues within the body, and is similar to the adult form of the disease.3 Younger children (<4 years old) present with a triad of the following symptoms:
- Arthritis (a condition characterised by inflammation [swelling], pain and stiffness in joints, affecting their function and movement)3
- Rash3
- Uveitis (inflammation of the middle layer of the eyes, and typically associated with redness, light sensitivity and visual disturbances)3
Diagnosis of sarcoidosis
Doctors may find it hard to diagnose sarcoidosis in children due to both the rarity of the disease and the symptoms being similar to those commonly seen in other conditions2. However, if a doctor suspects sarcoidosis in an individual, they will ask about the family’s medical history and request some tests to be carried out.
One standard diagnostic method is to conduct a biopsy of the lung (or other affected organ), which is a medical procedure where a small sample of the tissue of interest is taken from the body and studied under a microscope to detect abnormalities. In sarcoidosis, a biopsy of the lung is taken to identify granulomas. This procedure is carried out by a surgeon under local anaesthetic, so the child will not feel any pain.2
A doctor may want to look inside the affected person’s lungs, and they can do this by passing a long, thin tube with a light source and camera mounted to one end (known as an endoscope) down the person’s throat.1 A small sample of lung tissue is collected during this procedure and examined under a microscope to identify granulomas.
A chest X-ray and CT scan can be conducted to examine the lungs and lymph nodes.2
The function of the child’s lungs can also be determined through breathing tests.2
Currently, there is no definitive laboratory test that can detect sarcoidosis.3 Tissue biopsy remains the standard method for a definitive diagnosis of the condition.
Treatment of sarcoidosis
In most individuals affected by sarcoidosis, symptoms improve without any medical intervention for months or years.1 The symptoms are also not as severe in these people. However, the symptoms can gradually worsen over time in some people, and this is called chronic sarcoidosis. Acute sarcoidosis happens in individuals who develop symptoms quickly but these clear within a couple of months or years and the condition does not return.1
The main aim of therapeutic interventions is to reduce inflammation and the development of granulomas that affect the normal functions of the organs they are found in.2
There is currently no definitive cure for sarcoidosis, but medicines can help manage symptoms.
The primary treatment options for children with sarcoidosis are:
- Steroid medication: to reduce inflammation (swelling)2
- Immunosuppressive medication: to suppress the immune system from overreacting2
- Physical therapy: to help the child maintain usage of their muscles and joints2
Steroids come in several forms, such as tablets, injections, nasal sprays and inhalers.4 Most steroids can only be accessed through a prescription but some (such as creams) may be purchased at a local pharmacy or shop. However, it is best to first seek advice from your healthcare provider. If the individual stops taking the steroid medication, their symptoms may come back, and in this case, they may be asked to restart their treatment.2 The side effects of steroid tablets, if taken in high doses, have been noted to be weight gain and changes in mood.1 The doctor may initially recommend the individual to take a high dose of steroid medicine for a short duration before they are put on a low dose regimen for the following months.1 The patient’s health will be monitored to determine the effect of the treatment.
Immunosuppressive medications such as methotrexate can be used to lower the immune system’s activity, however this may affect the normal ‘defending function’ of the immune system and make an individual more prone to infections, so it is important to discuss the benefits and risks of this medication with a healthcare provider.5
Management of sarcoidosis
The management of sarcoidosis typically involves monitoring the symptoms to evaluate the effectiveness of the treatments. Sarcoidosis can affect multiple organs, therefore it may be beneficial for the patient to have a multidisciplinary team assist them in managing their condition. Medications are often prescribed to help reduce symptom severity and prevent the onset of complications.
FAQs
Does sarcoidosis worsen with age?
Sarcoidosis can vary greatly among affected individuals. It may remain stable or improve over time in some people, but in others, it can progress or worsen. Patients aged 65 and over may experience a poorer prognosis.
What lifestyle measures are recommended for someone with sarcoidosis?
Some lifestyle changes that may help someone with sarcoidosis include eating a balanced diet, stopping smoking, maintaining a healthy weight, sleeping regularly and exercising.
What is the life expectancy of sarcoidosis patients?
Most people with sarcoidosis have a normal life expectancy.
How to tell if sarcoidosis is active?
Sarcoidosis is generally difficult to diagnose because the symptoms are similar to many other conditions. In general, symptoms of concern that may prompt someone to seek medical advice from their healthcare provider include fatigue, fever, swollen lymph nodes and weight changes.
Summary
Sarcoidosis is a rare condition in children that occurs when the body’s immune system mistakenly overreacts to an environmental trigger, resulting in inflammation and the consequent formation of granulomas. The organs typically affected are the lungs, lymph nodes and skin. Sarcoidosis may affect multiple organs at a time, and if this is the case, the affected individual may require management of their condition from a multidisciplinary team. Some symptoms associated with the condition include fever, fatigue and weight changes, but these are general symptoms and the patient will need to be examined by their doctor to determine what appropriate diagnostic course of action should be carried out. The standard diagnostic method is a lung biopsy. Other methods include a chest X-ray and CT scan. Most children with sarcoidosis do not require treatment as the condition improves by itself. However, those with persistent or worsening symptoms may be recommended steroids or immunosuppressive medications to help reduce inflammation and stop the immune system from overreacting.
References
- Sarcoidosis. nhs.uk [Internet]. 2017 [cited 2024 May 31]. Available from: https://www.nhs.uk/conditions/sarcoidosis/.
- Sarcoidosis in children | Asthma + Lung UK [Internet]. 2022 [cited 2024 May 31]. Available from: https://www.asthmaandlung.org.uk/conditions/sarcoidosis/children.
- Pediatric Sarcoidosis: Practice Essentials, Background, Etiology [Internet]. 2022 [cited 2024 May 31]. Available from: https://emedicine.medscape.com/article/1003964-overview?form=fpf.
- Steroids. nhs.uk [Internet]. 2017 [cited 2024 May 31]. Available from: https://www.nhs.uk/conditions/steroids/.
- Association AL. Diagnosing and Treating Sarcoidosis [Internet]. [cited 2024 May 31]. Available from: https://www.lung.org/lung-health-diseases/lung-disease-lookup/sarcoidosis/treating-and-managing.