F. Merkel et al., THERAPEUTIC OPTIONS FOR CRITICALLY ILL PATIENTS SUFFERING FROM PROGRESSIVE LUPUS NEPHRITIS OF GOODPASTURES-SYNDROME, Kidney international, 1998, pp. 31-38
Systemic lupus erythematosus is a chronic disease with many clinical f
eatures, while Goodpasture's syndrome usually becomes manifest with pr
ogressive glomerulonephritis and pulmonary hemorrhage. Rapidly declini
ng renal function and even pulmonary hemorrhage may be the common feat
ure. Early and precise diagnosis is most important as it may provide g
eneral prognostic information and serve as a guideline for initial the
rapy. Immunosuppression with oral cyclophosphamide and high dose corti
costeroids together with plasmapheresis is used in Goodpasture's syndr
ome. Progressive lupus nephritis requires high dose corticosteroids to
gether with i.v. pulses of cyclophosphamide for at least six months, f
ollowed by maintanance immunosuppression. The benefits of therapy must
always be weighed against the risks. Nevertheless, current therapy re
mains less than optimal. A better understanding of the pathogenesis of
systemic lupus erythrematosis (SLE) and Goodpasture's syndrome may pr
ovide more specific information about the nature and the role of the i
mmune response and thus lead to new treatment strategies.