Therapeutic options for critically ill patients suffering from progressive lupus nephritis or Goodpasture's syndrome

Systemic lupus erythematosus is a chronic disease with many clinical features, while Goodpasture's syndrome usually becomes manifest with progressive glomerulonephritis and pulmonary hemorrhage. Rapidly declining renal function and even pulmonary hemorrhage may be the common feature. Early and precise diagnosis is most important as it may provide general prognostic information and serve as a guideline for initial therapy. Immunosuppression with oral cyclophosphamide and high dose corticosteroids together with plasmapheresis is used in Goodpasture's syndrome. Progressive lupus nephritis requires high dose corticosteroids together with i.v. pulses of cyclophosphamide for at least six months, followed by maintenance immunosuppression. The benefits of therapy must always be weighed against the risks. Nevertheless, current therapy remains less than optimal. A better understanding of the pathogenesis of systemic lupus erythrematosis (SLE) and Goodpasture's syndrome may provide more specific information about the nature and the role of the immune response and thus lead to new treatment strategies.