ABSTRACT
Reports on familial interstitial lung disease [ILD] are rare and all are indicative of high mortality. We report familial ILD in four siblings and their father. Lung biopsy on each patient revealed different stages of ILD in the form of usual interstitial pneumonia [UIP] in one child, desquamative interstitial pneumonia in 2 children, and non-specific interstitial pneumonitis with a predominance of inflammatory cells in another child. All four siblings presented with repeated chest infection, cyanosis and became oxygen dependent. Chloroquine was used in treating all 4 siblings with variable response. The child with UIP died from progressive disease while on treatment. The father was diagnosed at 40 years of age with ILD and extensive fibrosis, but did not respond to cyclophosphamide treatment. In conclusion, familial interstitial lung disease can present with different pathological stages in the same family members and the response to treatment depends on the degree of inflammation or fibrosis. The condition should be diagnosed early before it reaches an irreversible stage