Granulomatous Disease

Published on 06/06/2015 by admin

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Last modified 06/06/2015

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30 Granulomatous Disease

Noninfectious granulomatous disease is rare in children, with the exception of Crohn disease (see Chapter 110). This group of diseases includes sarcoidosis, granulomatous vasculitides (Wegener granulomatosis [WG] and Churg-Strauss syndrome [CSS]), and familial juvenile systemic granulomatosis (Blau syndrome).

Sarcoidosis

Sarcoidosis is an uncommon multisystem disorder of uncertain etiology involving granulomatous infiltrates in affected organs. Sarcoidosis occurs in children worldwide without gender predominance. The incidence ranges from 0.22 to 0.9 per 100,000 children in Denmark and Japan, respectively. Race predilection follows the predominant race of the country (e.g., whites in Scandinavian countries and Asians in Japan); however, there is a higher prevalence in 8- to 15-year-old black children of the southeastern United States. The incidence in children in the United States is unclear owing to lack of systematic evaluation.

Clinical Presentation

Sarcoidosis in children occurs in two distinct forms. Older children (age 8-15 years) typically present with symptoms resembling the adult form of disease, including lymphadenopathy, lung involvement (often asymptomatic), systemic signs (fever, weight loss), and hypercalcemia, but rarely with joint involvement. In children younger than 5 years, the onset typically consists of the triad of rash, arthritis, and uveitis. Sarcoidosis commonly presents with lymphadenopathy and may affect a wide range of organs (Figure 30-2).