Pediatric sarcoidosis with diagnostic and therapeutical insights

Sarcoidosis is a chronic granulomatous disorder involving multiple systems and organs of undefined etiology. Although most of the morbidity relies upon lung disease, the function of several systems and organs can be affected. The natural history of lung disease consists of pulmonary involvement. An exaggerated and abnormal inflammatory response accompanies this aspect. There are noncaseating confluent epithelioid granulomas and, potentially, a progressive airway obstruction ab externo. As the disease is multisystemic, there is an increased likelihood of complications that may be serious and even fatal.

The American Thoracic Society (ATS) Core Curriculum updates clinicians annually in adult and pediatric lung disease, critical medical care, and sleep medicine. In late 2020, the ATS targeted sarcoidosis. Also, in 2019, the French Sarcoidosis Group thoroughly revised the literature on pediatric sarcoidosis. Currently, staging is based on chest radiograph findings, and the most commonly used system is the Scadding classification, which has been applied to both children and adults alike. Treatment may consist of oral or pulsed intravenous corticosteroids, but it should be implemented in union with a rheumatologist, as there are no randomized controlled studies in children.

Sarcoidosis is rare in childhood. Diagnosis is complex and relies on multiple diagnostic modalities with both staging and therapy progressively mirroring the sarcoidosis, which affects adults. In the majority of patients, spontaneous resolution will occur and observation is justified above treatment. Nevertheless, in case treatment is needed corticosteroids remain the mainstay of the treatment in some pediatric patients. Relapses are not uncommon and a long-term follow-up is essential.