Development of progressive pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG) associated with therapy-resistant chronic systemicjuvenile arthritis (CJA)

A girl aged 5 years with therapy-resistant chronic systemic juvenile arthri tis (CJA) developed progressive fibrosing lung disease. Histology of an ope n lung biopsy revealed pulmonary interstitial and intra-alveolar cholestero l granulomas (PICG). Since treatment with steroids and immunosupressive drugs did not prevent pr ogression of lung fibrosis, an experimental treatment with a tumor necrosis factor alpha (TNF alpha) antagonist etanercept was started. Although devel opment of chronic changes in the lung parenchyma could not be prevented, th is treatment brought considerable relief and markedly improved the child's physical capacity. By ruling out other causes for development of PICG, we c oncluded that the primary disease had caused the development of cholesterol granulomata by macrophage activation. We suggest, therefore, that a trial with etanercept in children with otherw ise therapy-resistant CJA should be considered, especially if pulmonary com plications have developed. (C) 2001 Wiley-Liss, Inc.