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Five of the six sufferers had proof interstitial lung disease, and all had hilar or paratracheal lymphadenopathy as observed on computed tomography of the upper body . However, Sufferers 3, 4, and 6 experienced no respiratory symptoms. Lung-biopsy samples from two individuals showed a scattered combined lymphocytic inflammatory infiltrate , interstitial fibrosis, and emphysematous adjustments . All of the patients had failing to thrive . By 7 years, Patient 5 had symmetric seropositive polyarthritis with carpal-bone erosions and progressive ulnar deviation . Two patients had histologically verified myositis and muscle mass atrophy . Both of these children died from pulmonary problems and secondary infections. An autopsy performed on Patient 2 showed a widespread vasculopathy of the systemic and pulmonary vasculature .