Sarcoidosis in children With special emphasis on the natural history and treatment

Summary Twenty-five cases of childhood sarcoidosis were diagnosed at North Carolina Memorial Hospital between 1954 and early 1968. Biopsies from 22 cases were compatible with the diagnosis of sarcoidosis; the other 3 cases had negative biopsies but their clinical courses were consistent with a positive diagnosis. Other chronic granulomatous diseases were ruled out by skin tests, appropriate cultures, special staining of biopsy material, and clinical course of the patients. The age, sex, and race distribution of the North Carolina patients was similar to that in other reported series. In the North Carolina series, the average age at diagnosis was 13 years, the male to female ratio was almost equal, and the Negro race predominated. Cases were scattered in the hospital's referral area in middle and eastern North Carolina and did not suggest any special epidemiological pattern. Weight loss, cough, and fever were the most frequent of an otherwise variable pattern of signs and symptoms. The frequency of signs and symptoms was higher than in reported series where specific data on symptomatology were not always indicated. In the North Carolina series there was multisystem involvement in 23 of 25 patients. All patients had pulmonary parenchymal and/or hilar node involvement and 12 patients had ocular disease. Lung, lymph node, eye, liver, spleen, parotid gland, nervous system, heart, and striated muscle involvement occurred more commonly in the North Carolina patients than in other reported cases. Hypercalcemia, hyperproteinemia, and eosinophilia were the most persistent abnormal routine laboratory findings and were observed in a higher percentage of the North Carolina patients than in reported cases. Eleven patients had pulmonary ventilatory studies performed and 6 were abnormal. Of the 18 patients who were followed for one year or more, 10 were treated with corticosteroids and 8 were treated symptomatically without corticosteroid therapy. In the nonsteroid-treated group, symptoms were variable and new ones tended to appear during follow-up. The degree of organ involvement at the onset in this group generally remained unchanged but in some patients there was progression to other organs. In the corticosteroid-treated group, in which all patients had ocular involvement and the disease was generally more severe, symptoms resolved within a few months. In most cases organ involvement resolved or was diminished in severity. It was uncommon for the disease to recur after corticosteroids were discontinued. Comparative data from the literature on the natural history and effect of corticosteroid therapy are not available. In middle and eastern North Carolina sarcoidosis in children would appear to be more common than previously suspected. The multiplicity of system involvement and the severity of the disease were evident in these patients. Although longer periods of observation are necessary and controlled studies need to be performed for a better evaluation of this disease, the data presented here suggest that corticosteroids are beneficial in alleviating symptoms and in resolving or decreasing the severity or organ involvement.