BACKGROUND: Side-effects, including autoimmune disorders, are frequent with
D-penicillamine therapy. Proteinuria is observed in 10% of the patients, o
ften secondary to extramembranous glomerulopathy. Necrotizing extracapillar
y glomerulonephritis is however exceptional.
CASE REPORTS: Two patients with systemic sclerodermia were treated with D-p
enicillamine for 7 and 14 years. Both developed necrotizing extracapillary
glomerulonephritis with anti-myeloperoxidase antibodies (anti-MPO), associa
ted with hemorrhagic alveolitis in one case. Partial regression of the rena
l failure was obtained after withdrawal of D-penicillamine and combination
treatment with prednisone and cyclophosphamide.
DISCUSSION: Extracapillary glomerulonephritis or a lung-kidney syndrome are
frequently associated with anti-MPO antineutrophil cytoplasm antibodies (A
NCA). In systemic sclerodermia, the presence of anti-MPO appears to define
a group of patients at risk of pauci-immune extracapillary glomerulonephrit
is or a lung-kidney syndrome. In addition, the presence of ANCA in patients
with renal failure would suggest extracapillary glomerulonephritis rather
than sclerodermic microangiopathy. Development of extracapillary glomerulon
ephritis with anti-MPO in patients who are taking D-penicillamine suggests
that inductor mechanisms other than D-penicillamine are involved in the pat
hogenesis of these glomerulopathies.