Sarcoidosis in Children

Sarcoidosis is a granulomatous inflammatory condition with multisystem involvement of unestablished etiology. A century has passed by since this condition was first described by Hutchinson, however there still remains a serious lack of awareness regarding this illness amongst the healthcare professionals involved in treating children.

Epidemiology:

The condition, although more common in the age group of 20 - 45 years, can very well involve children of any age. It is feared that due to lack of awareness and nature of the illness, cases may remain undiagnosed or misdiagnosed. Added to this is the paucity of large scale epidemiological studies in children, that has resulted in underestimation of actual prevalence and incidence in the community. Data available from India is largely that of adult population. Worldwide data suggests that the disease is influenced by racial background and geographical location in terms of prevalence, clinical presentation and progression of illness. In one of the recent studies done in Denmark, where the prevalence rate is known to be high, incidence reported in children aged 14-15 years was 1.02 per 100000 and 0.06 per 100000 in children under 4 years of age.

Etiopathogenesis:

Etiopathogenesis is not completely understood, but exaggerated cellular immunity is considered to be central in the pathogenic process leading to granuloma formation. In a genetically predisposed individual, pathogenic and non-pathogenic environmental antigen triggers a cascade of inflammatory responses with release of excessive chemokines, cytokines and interferons that interact to form granuloma and fibrosis. Persistence of granulomas perpetuate further inflammatory process. Significant role of MHC class II molecules in the...

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...hamide, ciclosporin and anti TNF alpha agents. There has also been mention in case reports of use of thalidomide and chloroquine or hydroxychloroquine in moderately severe disease.

Guarded prognosis is often given when the disease starts early in life. Late onset disease has variable prognosis and depends on various factors including race and geographical location. The disease may show spontaneous remission in some. Recurrences of the disease after remission has also been noted and hence long term follow up of patients after remission is advisable. Often the overall prognosis is felt to be generally good with timely instituted treatment. However the first step in treating depends on prompt recognition and diagnosis of the illness. The popular saying - ‘Eyes cannot see what the mind does not know’ applies well to this challenging and difficult to diagnose condition.