Idiopathic Pulmonary Hemosiderosis

Abstract
Idiopathic pulmonary hemosiderosis or essential brown induration of the lung is a rare disease occurring in children. Ceelen (1), in 1921, first recognized this entity and described the morbid anatomy. Further important contributions were made by Anspach (2) in 1939, with the first roentgenologic observation, and by Waldenström (3) in 1944, with the first roentgenologic-clinical-pathological correlation. Wyllie, Sheldon, Bodian, and Barlow (4) in 1947 collected 17 cases from the literature, added 7 new observations, and surveyed the clinical, radiologic, and hematologic features and the morbid anatomy. It appears that 31 cases of the disease have been reported, 4 of them from America (2, 5, 6, 7). Five additional cases in the age range of nineteen to thirty years were mentioned by Kerley (8), but the description is too scanty for their inclusion here.2 The disease usually starts in early childhood. One case (9) has been reported in an infant of four months. With the single exception of a man of thirty-eight (10), no patient was older than sixteen years. There have been 19 female and 10 male patients, the present case included. In the 3 remaining cases the sex is not known. There appears to be no hereditary or familial predisposition. In only 1 case (11) was there a suspicion in this direction, repeated hemoptyses having occurred in the patient's father. There is no apparent geographic or racial influence. Our observation seems to be the first in a Negro child. The duration of the disease was one to three years in most of the cases; the shortest was five weeks in a girl of two and a half years. The course is usually intermittent and progressive, and the earlier writers invariably reported a fatal outcome. In the series of Wyllie et al. (4), however, 4 patients, were alive at the time of the report, some eight and nine years after the onset of the disease. The child observed by Elgenmark and Kjellberg (12, 13) is now fourteen years old and in good general health, at least twelve years after onset of pulmonary hemorrhages. There is reasonable possibility that non-fatal cases have escaped recognition in the past and that the prognosis is not as unfavorable as it seemed to be. The disease manifests itself usually by rather sudden attacks of fatigue, tachycardia, and dyspnea. Pallor or cyanosis is usually present. Cough, often accompanied by hemoptysis, and vomiting with hematemesis may occur; occult blood has been found in the stools. There may be acute right heart failure with engorged neck veins, gallop-rhythm, and enlargement of liver and spleen. Fever is not uncommon. These attacks may last a few days or weeks and are followed by relatively symptom-free intervals. Most of these episodes are the clinical manifestations of massive and extensive diapedesis of red blood cells from the pulmonary capillary vessels. Eventually a severe anemia of the iron-deficiency type develops.