%0 Journal Article
%T Childhood sarcoidosis: A rare but fascinating disorder
%A Avinash K Shetty
%A Abraham Gedalia
%J Pediatric Rheumatology
%D 2008
%I BioMed Central
%R 10.1186/1546-0096-6-16
%X Sarcoidosis is a multisystem systemic granulomatous disease of unknown etiology that most commonly affects young adults, who frequently present with hilar lymphadenopathy, pulmonary infiltration, and ocular and cutaneous lesions [1]. Although the lung is most frequently involved, the disease can affect any organ system of the body [2]. The disease is relatively rare in the pediatric population [3,4]. Infants and children younger than 5 years usually present with the triad of skin, joint, and eye involvement, without typical lung disease. However, older children have involvement of the lungs, lymph nodes, and eyes more frequently, as seen in adult [5,6].Despite a variety of hypotheses regarding causative agents, the cause of sarcoidosis is unknown [7]. The definitive diagnosis of sarcoidosis is made when compatible clinical findings are associated with histopathological evidence of noncaseating granulomata in affected organs and other granulomatous disorders are excluded [8]. The illness can be self-limited or chronic, and the disease characteristics vary among various populations [1]. The course and prognosis of sarcoidosis in children is different compared to adults, and may correlate with the mode of onset and the extent of the disease [1,9,10].In this review, we discuss the epidemiology, pathogenesis, etiology, and clinical features of sarcoidosis in children, and examine the current approaches to diagnosis and treatment of this enigmatic disease.The prevalence of sarcoidosis in the adult population ranges from 10 to 40 per 100,000 in the United States and Europe [1]. The true incidence and prevalence of childhood sarcoidosis is unknown because of the rarity of the disease and the small number of reported cases in childhood. A recent review reported that the approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 years was 0.22¨C0.27 per 100,000 children per year, corresponding to approximately 3 new cases in Denmark each year
%U http://www.ped-rheum.com/content/6/1/16