Friday, March 29, 2024

Pediatric Sarcoidosis

Practice Essentials

Sarcoidosis is a systemic granulomatous disease that is relatively rare in children. The diagnosis is established when histopathologic evidence of noncaseating granulomata in affected organs and exclusion of other granulomatous diseases support compatible clinical and radiographic findings (see the image below). Glucocorticoids are the treatment of choice for children with multisystem involvement.

Chest radiograph showing bilateral hilar lymphaden

Chest radiograph showing bilateral hilar lymphadenopathy in a 10-year-old girl with sarcoidosis.

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Signs and symptoms of pediatric sarcoidosis

The presentation in sarcoidosis can vary widely, depending on the extent and severity of organ involvement. In most children, the disease frequently involves the lungs, lymph nodes, eyes, skin, liver, and spleen.

Two distinct forms of childhood sarcoidosis are noted. Older children typically present with a multisystem disease similar to the adult manifestation, with frequent lymphadenopathy and pulmonary involvement, as well as generalized signs and symptoms, such as fever and malaise. In contrast, early onset childhood sarcoidosis occurs in patients who are younger than age 4 years and is characterized by the following triad:

Rash

Uveitis

Arthritis

See Presentation for more detail.

Diagnosis of pediatric sarcoidosis

Imaging studies

Imaging studies used in the workup of pediatric sarcoidosis include the following:

Chest radiography

High-resolution computed tomography

Gallium-67 scanning

Laboratory studies

No definitive laboratory test diagnostic of sarcoidosis has been identified.

Biopsy

Tissue biopsy is required for a definitive diagnosis. Biopsy specimens should be obtained from the most readily accessible organ with the least invasive method.

See Workup for more detail.

Management of pediatric sarcoidosis

The goal of therapy in sarcoidosis is to prevent or minimize inflammation and granuloma formation leading to organ system dysfunction, which may ultimately cause end-stage organ destruction by the development of hyaline fibrosis. Glucocorticoids remain the therapy of choice for children who have multisystem involvement.

See Treatment and Medication for more detail.

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