Nephrology Dialysis Transplantation, Vol 13, Issue 4 935-939, Copyright © 1998 by Oxford University Press
C Ang, J Savige, J Dawborn, P Miach, W Heale, B Clarke and R Sinclair
Background: This study compared the clinical and
laboratory characteristics of patients with antiglomerular basement
membrane (GBM) disease and normal renal function, with those of patients
with anti-GBM disease where there was renal impairment.
Methods: The medical records of the 14 patients who
had presented with anti-GBM disease to our hospital in the past 20 years
were reviewed. Results: Five (36%) had a normal serum
creatinine or creatinine clearance at presentation. Other features were
haemoptysis (2/5, 40%), macroscopic haematuria (2/5, 40%) or systemic
symptoms (1/5, 20%). All five (100%) had some degree of haematuria, four
(80%) had proteinuria of at least 1 g/day, and none was hypertensive.
Anaemia, raised WCC, or elevated ESR (<35 mm/h) occurred less often
than in patients with impaired renal function (P <0.05). Two of the
five (40%) with normal renal function had circulating anti-GBM antibodies,
which were present at low or moderate levels; but seven of the nine with
renal impairment (77%) had circulating antibodies, with high levels in
five. Renal biopsies from patients with normal renal function were normal
(1/5, 20%), showed mesangial proliferation (4/5, 80%) or had more than 20%
glomeruli sclerosed (1/55, 20). Complement deposition was present in 2/5
biopsies (50%). The kidneys from patients with renal impairment had
crescents in more than 50% glomeruli (9/9, 100%, and four had more than 20%
glomeruli sclerosed (44%). All four kidneys from patients with renal
impairment that were examined had complement deposits (100%). Treatment was
identical in both groups; patients with normal renal function were followed
for a median of 48 months, and those with renal impairment for 180 months.
There were no further episodes of haemoptysis, haematuria, or other
symptoms of relapse in either group. All five patients with normal renal
function are alive, and the serum creatinine is less than 0.2 mmol/l in all
(100%), but haematuria persists in one (20%), and proteinuria <1
g/day in two (40%). Eight of the nine (89%) patients with impaired renal
function survive, but all are currently being dialysed or have had a renal
transplant. Conclusion: Patients with anti-GBM disease
with normal renal function are not uncommon, and often have a good
prognosis. There is less renal damage, possibly because of lower levels of
circulating anti-GBM antibodies and less glomerular complement deposition.
Key words: anti-GBM antibodies; anti-GBM disease;
creatinine; kidney function; renal failure
ORIGINAL ARTICLES
Antiglomerular basement membrane (GBM)-antibody-mediated disease with normal renal function
Renal Unit, University Department of Medicine, and Department of Anatomical Pathology, Austin and Repatriation Medical Centre, Heidelberg, Victoria, Australia 3084; Corresponding author
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