M. Coleman et al., GLOMERULAR-BASEMENT-MEMBRANE THINNING IN A PATIENT WITH HEMATURIA ANDHEMOPTYSIS MIMICKING GOODPASTURES-SYNDROME, American journal of nephrology, 14(1), 1994, pp. 47-54
Ultrastructural morphometric studies of glomerular basement membrane (
GBM) thickness are described in two renal biopsy specimens from a pati
ent who presented with hemoptysis and hematuria mimicking Goodpasture'
s syndrome. Significant GBM abnormality, with attenuation as the main
lesion, identified in a biopsy specimen taken during active clinical d
isease appeared to have resolved in a second biopsy specimen taken dur
ing the recovery phase. There was no evidence of glomerulonephritis. C
oncurrent lung biopsy studies showed focal alveolar-capillary wall bas
al lamina changes of uncertain diagnostic significance. These observat
ions suggest the alternative possibilities that GBM attenuation may be
either an acquired consequence of systemic disease or may be part of
an hitherto unrecognized primary multisystem abnormality of basal lami
na affecting, in this case, glomerular and pulmonary laminae, resultin
g in hematuria and hemoptysis. The morphometric studies in this case i
ndicate that simple-mean measurements of GBM thickness are inadequate
alone for the quantitative study of this lamina because significant in
terand intraglomerular membrane variation, if irregularly distributed,
can remain undetected.