We report on a family (mother, daughter, and son) suffering from progressive pulmonary fibrosis associated with deforming arthritis, sinusitis, glomerulonephritis, and cutaneous vasculitis. We suggest that these clinical features display the variable expressions of small-vessel vasculitis in juvenile rheumatoid arthritis.
Pulmonary hypoplasia is a rare congenital anomaly, which is frequently associated with other congenital anomalies. Most patients have recurrent episodes of wheezing or pneumonia and severe respiratory distress leading to chronic respiratory failure whereas some may be asymptomatic. Although it is usually diagnosed in infancy and childhood, some cases do not show any symptoms until the adolescent ages. In adolescents and adults with unilateral hypolucent lung, pulmonary hypoplasia should always be kept in mind.
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