Sarcoidosis is a disease that is mainly diagnosed in adults from the ages of 20-40. It is characterized by the formation of clumps of immune cells called granulomas in different tissues and organs throughout the body.
Childhood sarcoidosis is extremely rare and its true prevalence is unknown. A Danish study estimates that 0.22-0.27 out of 100,000 children develop sarcoidosis, often in their early teens.
Types of childhood sarcoidosis
Childhood sarcoidosis is a systemic disease with variable worsening depending on the age of onset. There are two distinct types of childhood sarcoidosis, early- and late-onset.
Early-onset childhood sarcoidosis develops in children younger than 4, while late-onset disease is typically seen in older children. Children with early-onset sarcoidosis commonly have three features, including a rash, arthritis, and uveitis (inflammation of the middle layer of the eye).
Children with late-onset sarcoidosis usually develop the disease when they are around 13-15, with multisystemic symptoms similar to those seen in adults. The respiratory system is most often affected.
Symptoms of childhood sarcoidosis
Late-onset childhood sarcoidosis is more common than early-onset disease. While the lungs are the most commonly affected organ, the disease can target any organ.
Children often have enlarged lymph nodes in the lungs, known as hilar lymphadenopathy, mucus in the lungs, and lesions in the eyes or skin. Hilar lymphadenopathy is more common in late-onset sarcoidosis and rare in early-onset disease, while uveitis is more common in early-onset sarcoidosis.
Early-onset sarcoidosis may be mistaken for juvenile rheumatoid arthritis, as both diseases are associated with nonspecific symptoms like weight loss, fever, and fatigue. Special attention to rashes on the skin can help doctors differentiate between the two conditions.
Additionally, arthritis is different between these two diseases; arthritis in sarcoidosis does not limit the child’s range of motion and is painless, while arthritis in juvenile rheumatoid arthritis is painful and impairs the child’s range of motion.
Diagnosis of childhood sarcoidosis
The diagnosis of childhood sarcoidosis is extremely difficult because it is variable and often appears like other diseases. A correct diagnosis depends on an assessment of the clinical symptoms and confirmation of the presence of granulomas by imaging or biopsy analysis, similar to tests done in adults.
Treatment of childhood sarcoidosis
Corticosteroids are the main form of treatment for children with sarcoidosis. Dosing and timing of treatment must be tailored for each patient. Treatment is continued until there’s a significant improvement, then the dose is gradually reduced.
Some children may experience deterioration after a period of successful treatment. When this happens, children may need to start taking the steroid again. In some cases, immunosuppressants or medications that suppress the activity of the immune system may be used instead of steroids or in combination with them.
Potential genetic component of childhood sarcoidosis
The exact cause of sarcoidosis, including the childhood type, is not known. Familial cases of sarcoidosis exist and hint at the presence of a genetic component as a possible cause of the disease.
For example, mutations in a gene called NOD2 have been associated with inherited childhood sarcoidosis. However, there is only limited data to support a potential link between childhood sarcoidosis and genetics, as many children with the disease do not have the NOD2 gene mutation.
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