GOODPASTURE'S SYNDROME AND WEGENER'S GRANULOMATOSIS

Abstract

SUMMARYThe case histories of six patients with Goodpasture's syndrome are contrasted with those of three patients with Wegener's granulomatosis. There is a similarity between the two, because each is initially a respiratory disorder complicated by glomerulonephritis. In each the glomerular lesions vary from deposits of fibrinoid material through exudative and proliferative reactions towards complete obliteration of all glomeruli. The pulmonary lesions, however, differ markedly. The typical lesion in Goodpasture's syndrome is hæmorrhage into the lungs giving rise eventually to pulmonary siderosis, whereas in Wegener's syndrome there is replacement of the lining of bronchi and of accessory nasal sinuses by necrotizing granulomatous tissue which may simulate carcinoma or tuberculosis. Widespread arteritis is a constant feature of Wegener's granulomatosis, but is seldom found in Goodpasture's syndrome. It was present in one of the six examples of Goodpasture's syndrome, but was of only minor importance.Although renal biopsy cannot distinguish between the two conditions, it is helpful in directing attention to these disorders. Biopsy of the upper air passages, however, may be diagnostic in Wegener's granulomatosis, as it was in two of the three cases in this survey.In one case of Goodpasture's syndrome, corticosteroids may have caused some mitigation of the disorder, but in the other five cases of Goodpasture's syndrome and in the case of Wegener's granulomatosis in which corticosteroids were used, any benefit was only temporary.