[Extracapillary glomerulonephritis with anti-myeloperoxidase antibodies in 2 patients with systemic scleroderma treated with penicillamine D].
BACKGROUND
Side-effects, including autoimmune disorders, are frequent with D-penicillamine therapy. Proteinuria is observed in 10% of the patients, often secondary to extramembranous glomerulopathy. Necrotizing extracapillary glomerulonephritis is however exceptional.


METHODS
Two patients with systemic sclerodermia were treated with D-penicillamine for 7 and 14 years. Both developed necrotizing extracapillary glomerulonephritis with anti-myeloperoxidase antibodies (anti-MPO), associated with hemorrhagic alveolitis in one case. Partial regression of the renal failure was obtained after withdrawal of D-penicillamine and combination treatment with prednisone and cyclophosphamide.


CONCLUSIONS
Extracapillary glomerulonephritis or a lung-kidney syndrome are frequently associated with anti-MPO antineutrophil cytoplasm antibodies (ANCA). In systemic sclerodermia, the presence of anti-MPO appears to define a group of patients at risk of pauci-immune extracapillary glomerulonephritis 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 glomerulonephritis with anti-MPO in patients who are taking D-penicillamine suggests that inductor mechanisms other than D-penicillamine are involved in the pathogenesis of these glomerulopathies.
Kyndt
X
X
Service de Médecine interne et Néphrologie, Centre Hospitalier de Valenciennes.
Ducq
P
P
Bridoux
F
F
Reumaux
D
D
Makdassi
R
R
Gheerbrant
J D
JD
Vanhille
P
P
D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001327:Autoimmune Diseases / Q000139:chemically induced; D005260:Female; D005921:Glomerulonephritis / Q000139:chemically induced / Q000276:immunology*; D006801:Humans; D008875:Middle Aged; D010396:Penicillamine / Q000009:adverse effects / Q000627:therapeutic use*; D011507:Proteinuria / Q000139:chemically induced; D011928:Raynaud Disease / Q000150:complications* / Q000276:immunology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D012737:Sex Factors