Paediatric sarcoidosis

Hypercalcemia, an Important Puzzle Piece in Uncommon Onset Pediatric Sarcoidosis—A Case Report and a Review of the Literature

“…Sarcoidosis (SD) or Besnier-Boeck-Schaumann disease was described for the first time in seventieth century as a systemic granulomatous condition of the lungs and lymph nodes (1). It is mainly a disease of the adults, with a peak onset between 25 and 45 years of age and with an incidence of ∼5-45 in 100,000 adults (2), whereas it is almost 10 times less common in children (3). Even though its pathogenesis remains unknown, the current hypothesis involves an association between genetic predisposition and the environmental factors that demonstrate the ability to trigger or enhance the inflammatory and granulomatous processes (3).…”

“…The patients with SD express a wide spectrum of clinical manifestations, ranging from a subclinical form affecting only two organs to a life-threatening multiorgan dysfunction (4). The most common symptoms reported in children were related to lung and mediastinal involvement; however, extrarespiratory manifestations were also frequently observed, such as eye impairment, hepatomegaly, splenomegaly, skin anomalies, peripheral lymphadenopathy, or arthralgia (3,5). Laboratory tests are not usually reliable for the diagnosis because they are not specific.…”

“…Laboratory tests are not usually reliable for the diagnosis because they are not specific. Nevertheless, certain laboratory findings may be associated with SD in children, such as elevated erythrocyte sedimentation rate, anemia, hypergammaglobulinemia, lymphocytosis or lymphopenia, thrombocytosis, increased levels of transaminases and serum angiotensin-converting enzyme (ACE), and hypercalcemia (3), which also suggest other infectious pathologies (6). The assessment of renal function is also essential because hypercalcemia could lead to renal insufficiency or nephrolithiasis (7,8).…”

“…Nevertheless, pathological examination of the biopsied organs that reveal non-caseating granulomas remains the gold standard for SD diagnosis. Lung tissue biopsies could be obtained through wedge transbronchial lung biopsy; however, surgical biopsies are also a potential alternative (3). Other commonly biopsied organs in SD patients are peripheral or mediastinal lymph nodes and skin lesions (11).…”

Management of Sarcoidosis, a Selection of Topical Items Updating

“…Pediatric sarcoidosis is an extremely rare disease with a 0.6–1.02/100,000 estimated incidence, but in the absence of international registers, the disease is probably under-reported [ 43 , 44 ]. Chauveau et al’s report on child–adult transition in sarcoidosis is the first one on this topic, based on a large study [ 45 ].…”