Sarcoidosis
Sarcoidosis is a multi-systemic non-necrotizing granulomatous disease which can present with hilar lymphadenopathy, pulmonary infiltration and ocular and cutaneous lesions [126]. Patients can present with involvement of one system or multi-systemic disease. The diagnosis is made when there is pathologic evidence of noncaseating granulomas in affected tissue and other causes of granulomatous disease have been excluded. CVID and IEIs should be evaluated for in a pediatric patient presenting with sarcoidosis. Elevated ACE levels may support the diagnosis, but this is not necessary for diagnosis. While the incidence of sarcoidosis is unknown in the pediatric population, it is considered to be rare – a Danish case series of children > 15 years reported an incidence of 0.22 to 0.27 per 100,000 children per year [127]. Radiographic findings described in pediatric sarcoidosis include bilateral hilar lymphadenopathy with and without pulmonary infiltrates, and nodules with ground glass opacities, and pleural and interlobular septal thickening [128-130]. Reported PFT alterations include hypoxemia, reduced vital capacity, decreased DLCO, and decreased dynamic lung compliance [128, 129].
Decisions around treatment of pulmonary sarcoidosis in adults are challenging, with no consensus around who should receive treatment, given that disease can stabilize or resolve without therapy. These decisions are more complicated still in the pediatric population, with no studies around which patients to treat and optimal length of treatment [129]. Corticosteroids, either daily oral steroids (0.5 to 2 mg/kg/day) or monthly IV pulse steroids are reported as the most-used treatment [128, 129]. We recommend treatment of sarcoidosis in pediatric patients with moderate to severe reduction in pulmonary function, progressive decline in lung function, significant or worsening radiographic disease, persistent or worsening symptoms, exercise desaturation, and/or multisystemic involvement.