Rheumatoid Disease Of the Lung and Cor Pulmonale

Rheumatoid disease of the lung, the chronic pulmonary manifestation of rheumatoid arthritis, is rare, and its occurrence in childhood is even rarer. Since the original description of the disease by Ellman and Ball 1 in 1948, only 31 proved cases have been reported, according to Doctor and Snider, 2 and only one of these was in a child. In most reported cases emphasis is placed on pulmonary insufficiency resulting from the chronic inflammatory and fibrosing reaction that is invariably present in this disease. Pulmonary arteritis is usually also noted histologically, but is rarely of sufficient severity to produce the major physiologic disturbance. The clinical course of severe pulmonary arteritis may be similar to that of primary pulmonary hypertension. 3 The following case is reported because it is believed to be the second recorded case in a child and the major symptoms were due to pulmonary arteritis rather than pulmonary insufficiency,