Idiopathic Pulmonary Haemosiderosis with Allergic Pulmonary Vasculitis

Abstract

During the past decade an increasing number of cases of idiopathic pulmonary haemosiderosis have been reported. The clinical findings and symptomatology of this disease have become so well recognized that it is frequently diagnosed ante mortem. Despite an increasing knowledge of the clinical aspects of this disorder, its aetiology remains obscure. Certain morphological features encountered in the present case merit description because they suggest an allergic aetiology. CASE HISTORYA white housewife aged 46 years was admitted to the Lockboume Air Force Base Hospital, Columbus, Ohio, with a history of haemoptysis, dyspnoea, and non-pleuritic pain in the right chest posteriorly for the past three to four weeks. She had had two episodes of haemoptysis, 18 and nine years before admission, both of which necessitated bronchoscopy. In each instance no source of bleeding was found. Physical examination revealed no cyanosis or clubbing of the digits. The lungs were normal to percussion and auscultation. Chest radiography showed bilateral perihilar infiltration. The temperature was 100°F.She had a leucocytosis of 10,650 with 5% eosinophils, a haemoglobin of 10-4 g. per 100 ml. and haematocrit of 30%/. The blood urea nitrogen, serum protein, serum calcium, and phosphate concentrations were within normal limits. Sputum cultures and studies for acid-fast bacilli were negative.On the fourth day in hospital the patient developed a spiking fever and slight dyspnoea. She then experienced a haemoptysis of 250 ml. of bright red blood after which she became dyspnoeic, tachypnoeic, and cyanotic. The haemoglobin dropped to 8-3 g. per 100 ml. Treatment with oxygen, intermittent positive pressure breathing, blood transfusions, streptomycin, lanatoside c (cedilanid), andlaterhydrocortisone failed to improve her condition. She died in severe dyspnoea on the eleventh day in hospital. The clinical diagnosis was possible pulmonary haemosiderosis.NECROPSY Both lungs were heavy (right 950 g., left 900 g.) and on cross section all lobes showed haemorrhagic consolidation (Fig. 1). There was no evidence of pulmonary vessel sclerosis or other anomalies, and the heart did not show right ventricular hypertrophy. The hilar lymph nodes were slightly enlarged and pinkish-brown on cross section.HISTOPATHOLOGY The alveoli were filled to varying degrees with erythrocytes and haemosiderin-laden macrophages that stained intensely for iron. Occasionally, the alveolar surfaces were covered by a membrane of fibrin in a manner reminiscent of hyaline membrane disease of the newborn. Fragmentation of the elastic fibres with iron incrustation and fibrosis was encountered in the alveolar septa and in the small to medium sized pulmonary arteries and arterioles.In the interstitial tissue giant cells of the foreign body type with phagocytosed fragments of elastic fibres (Figs. 2A and 2B) were present in addition to deposits of iron. The capillaries and arterioles were engorged with blood. The endothelial cells were swollen and showed evidence of proliferation. Marke...