No abstract

The pathologic features of 29 cases of Goodpasture's syndrome occurring during a 13-yr period in Auckland have been reviewed and correlated with clinical findings. There were 20 males and nine females in the series; two of the males and three of the females were Maoris. Age at the time of onset of symptoms ranged from 17 to 75 yr, with about 76% of the patients being from 17 to 27 yr of age. Sixteen (55%) of the patients died from less than a week up to about two years following the onset of symptoms, and the remaining 13 are live from 30 weeks to 14 yr after initial presentation. Underlying renal disease varied from mild focal glomerulitis to end-stage glomerulonephritis by light microscopy, but characteristic glomerular changes were seen in all specimens examined by electron and immunofluorescent microscopy. The lungs of 13 of the patients examined at autopsy showed typical abnormalities. The syndrome pursues a notably variable clinical course, affects a considerable proportion of females, occurs over a wide age range, and appears to be disporportionately common among Maoris.

No abstract

No abstract

The clinical course and levels of anti-glomerular basement membrane (GBM) antibody were compared in 20 patients with Goodpasture’s syndrome treated with plasma exchange and immunosuppression (8 patients), immunosuppression alone (4 patients) or no specific therapy (8 patients). There was a more rapid fall in the level of anti-GBM antibody and pulmonary hemorrhage was less protracted in the 8 patients treated with plasma exchange and immunosuppression. In this group, 1 patient who presented with severe renal failure showed a marked improvement of renal function and there was no progression of disease in the 4 with milder renal involvement. 2 of the 4 patients treated with immunosuppression alone, and only 2 of the 8 patients who received no specific therapy, maintained normal renal function. In the group which received no specific therapy, 1 of the 6 patients who progressed to renal failure had mild renal involvement initially. There was a significant correlation between the level of anti-GBM antibody and the severity of the morphological changes seen at renal biopsy but not between the level of anti-GBM antibody and the severity of lung hemorrhage. The course and outcome of the disease in those patients not treated, or treated with immunosuppression alone, was better than that described in early reports of this disease, while those patients with plasma exchange and immunosuppression fared even better. An adequately stratified controlled trial of immunosuppression and plasma exchange versus immunosuppression alone is in order.